Swallowing kinematics and submental muscles activation during a newly designed maneuver called Mouth Open Swallowing Maneuver: A comparative study.

The aim of this study was to design a new maneuver called the Mouth Open Swallowing Maneuver (MOSM), and to compare swallowing kinematics and submental muscles activation (SMA) between MOSM and two current approaches used in dysphagia rehabilitation. Fifty healthy volunteers were asked to perform three repetitions of dry swallowing (DS) (control task), the MOSM, the Mendelsohn Maneuver (MM), and the Tongue-Hold Maneuver (THM) during videofluoroscopic swallowing study accompanied with simultaneous SMA recording. Swallowing kinematics were measured by frame-by-frame analysis on hyolaryngeal movement using ImageJ. Swallowing with maximum hyolaryngeal movement and SMA during these tasks was used for comparative analysis. Vertical movement of the hyoid during the MOSM was significantly greater than those observed during the DS and the THM (p<0.001, p<0.001). Horizontal movement of the hyoid during DS and the THM was significantly greater than that observed during the MM (p = 0.001, p = 0.001). Vertical movement of the larynx during the MOSM was significantly greater than those observed during DS, MM, and THM (p<0.001). There was no significant difference between tasks in horizontal movement of the larynx (p = 0.785). SMA during the THM was significantly greater than that observed during MOSM (p = 0.002). No significant difference was found between other tasks in terms of SMA (p>0.05). The MOSM as a newly designed maneuver was significantly superior to other maneuvers in increasing vertical hyolaryngeal movement. The THM has as much effect on hyolaryngeal movement as the MM. In this study, the MOSM was shown to be effective in increasing hyolaryngeal movement. ClinicalTrials.gov Protocol Registration and Results System (PRS); the clinical trial registration number (NCT05579041).

Radiomics in differential diagnosis of Wilms tumor and neuroblastoma with adrenal location in children.

OBJECTIVES: Machine learning methods can be applied successfully to various medical imaging tasks. Our aim with this study was to build a robust classifier using radiomics and clinical data for preoperative diagnosis of Wilms tumor (WT) or neuroblastoma (NB) in pediatric abdominal CT. MATERIAL AND METHODS: This is a single-center retrospective study approved by the Institutional Ethical Board. CT scans of consecutive patients diagnosed with WT or NB admitted to our hospital from January 2005 to December 2021 were evaluated. Three distinct datasets based on clinical centers and CT machines were curated. Robust, non-redundant, high variance, and relevant radiomics features were selected using data science methods. Clinically relevant variables were integrated into the final model. Dice score for similarity of tumor ROI, Cohen's kappa for interobserver agreement among observers, and AUC for model selection were used. RESULTS: A total of 147 patients, including 90 WT (mean age 34.78 SD: 22.06 months; 43 male) and 57 NB (mean age 23.77 SD:22.56 months; 31 male), were analyzed. After binarization at 24 months cut-off, there was no statistically significant difference between the two groups for age (p = .07) and gender (p = .54). CT clinic radiomics combined model achieved an F1 score of 0.94, 0.93 accuracy, and an AUC 0.96. CONCLUSION: In conclusion, the CT-based clinic-radiologic-radiomics combined model could noninvasively predict WT or NB preoperatively. Notably, that model correctly predicted two patients, which none of the radiologists could correctly predict. This model may serve as a noninvasive preoperative predictor of NB/WT differentiation in CT, which should be further validated in large prospective models. CLINICAL RELEVANCE STATEMENT: CT-based clinic-radiologic-radiomics combined model could noninvasively predict Wilms tumor or neuroblastoma preoperatively. KEY POINTS: • CT radiomics features can predict Wilms tumor or neuroblastoma from abdominal CT preoperatively. • Integrating clinic variables may further improve the performance of the model. • The performance of the combined model is equal to or greater than human readers, depending on the lesion size.

Childhood interstitial lung disease in Turkey: first data from the national registry.

The childhood interstitial lung diseases (chILD) Turkey registry (chILD-TR) was established in November 2021 to increase awareness of disease, and in collaboration with the centers to improve the diagnostic and treatment standards. Here, the first results of the chILD registry system were presented. In this prospective cohort study, data were collected using a data-entry software system. The demographic characteristics, clinical, laboratory, radiologic findings, diagnoses, and treatment characteristics of the patients were evaluated. Clinical characteristics were compared between two main chILD groups ((A) diffuse parenchymal lung diseases (DPLD) disorders manifesting primarily in infancy [group1] and (B) DPLD disorders occurring at all ages [group 2]). There were 416 patients registered from 19 centers. Forty-six patients were excluded due to missing information. The median age of diagnosis of the patients was 6.05 (1.3-11.6) years. Across the study population (n = 370), 81 (21.8%) were in group 1, and 289 (78.1%) were in group 2. The median weight z-score was significantly lower in group 1 (- 2.0 [- 3.36 to - 0.81]) than in group 2 (- 0.80 [- 1.7 to 0.20]) (p < 0.001). When we compared the groups according to chest CT findings, ground-glass opacities were significantly more common in group 1, and nodular opacities, bronchiectasis, mosaic perfusion, and mediastinal lymphadenopathy were significantly more common in group 2. Out of the overall study population, 67.8% were undergoing some form of treatment. The use of oral steroids was significantly higher in group 2 than in group 1 (40.6% vs. 23.3%, respectively; p = 0.040).   Conclusion: This study showed that national registry allowed to obtain information about the frequency, types, and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. What is Known: • Childhood interstitial lung diseases comprise many diverse entities which are challenging to diagnose and manage. What is New: • This study showed that national registry allowed to obtain information about the frequency, types and treatment methods of chILD in Turkey and helped to see the difficulties in the diagnosis and management of these patients. Also, our findings reveal that nutrition should be considered in all patients with chILD, especially in A-DPLD disorders manifesting primarily in infancy.

Esophageal Carcinoma in Children: Report of 2 Cases and a Review of the Literature.

Esophageal carcinoma in children and adolescents is extremely rare. Here, we report 2 cases of pediatric esophageal carcinoma presenting with progressive dysphagia. There was not any underlying specific risk factor in our cases. The histopathological subtypes were adenocarcinoma in one and squamous cell carcinoma in another case. Response to combined modality treatment was good in the case of adenocarcinoma, while the patient with squamous cell carcinoma was unresponsive to treatment and died of the progressive disease. We reviewed the pediatric cases of esophageal carcinoma reported in the literature. Progressive dysphagia was observed in 89% of these cases. One third of pediatric cases had underlying risk factors. Squamous cell carcinoma is a more common type of childhood esophageal carcinoma. In contrast to adults, pediatric esophageal squamous cell carcinoma may distribute throughout the esophagus. Esophageal adenocarcinoma was seen in the distal esophagus in pediatric cases. Metastatic disease was found in 48% of pediatric patients at presentation, and the prognosis is poor. Collaborative efforts are needed for success in the treatment of esophageal carcinoma.

Focal fatty sparing areas of the pediatric steatotic liver: pseudolesions on hepatobiliary phase magnetic resonance images.

PURPOSE: Focal fatty sparing in liver can be detected as hyperintense pseudolesions on hepatobiliary phase magnetic resonance imaging (MRI). Distinguishing these pseudolesions from liver lesions may make diagnosis challenging. The aim of this study was to evaluate the imaging features of fatty sparing areas on liver MRI in pediatric patients who have been administered gadoxetate disodium. METHODS: A total of 63 patients between January 2018 and June 2023 underwent gadoxetate disodium-enhanced liver MRI, and 9 (14%) patients with a focal fatty sparing were included in the study. The fat spared areas were evaluated qualitatively and quantitatively including signal intensity measurements and fat fraction calculations. RESULTS: The liver MRI examinations of 9 patients (5 boys, 4 girls; aged 8-18 years, median age: 14.4) using gadoxetate disodium were evaluated. Based on in-phase and opposed-phase sequences, 13 areas of focal fatty sparing were identified. The mean fat fraction of the liver and fat spared areas were 26.2% (range, 15-47) and 9% (range, 2-17), respectively. All fat spared areas were hyperintense in the hepatobiliary phase images. The mean relative enhancement ratios of the liver and fat spared areas were 0.78 (range, 0.35-1.6) and 1.11 (range, 0.45-1.9), respectively. CONCLUSION: Focal fatty sparing in liver in children was observed as hyperintense on hepatobiliary phase MRI, and it should not be identified as a focal liver lesion.

Ganglioneuromas in Childhood: Hacettepe Experience With 70 Cases.

BACKGROUND: Ganglioneuromas (GNs) are rare benign peripheral neuroblastic tumors (PNTs). We shared our institutional experience with childhood GNs. METHODS: Records of the children with PNTs between January 1995 and December 2021 were reviewed, and cases with histopathological diagnoses of GN were identified. Clinical, laboratory, radiological, and histopathological findings, image-defined risk factors (IDRFs), procedures, and overall outcomes were recorded. RESULTS: Of 668 cases with PNTs, 70 (10.4%) had GNs. The median age was 7.4 years (range, 2.6-15.7 years) (girls/boys, 41/29). Common presenting complaints were abdominal pain and cough; 33/70 cases (47.1%) were diagnosed incidentally. Primary tumors were in the abdomen in 41/70, the thorax in 25/70, the neck in 3 cases, and the pelvis in one. The median tumor size was 6.5 cm (range, 1.4-17). Fifty cases (71.4%) were staged as INRG-L1; 20 cases with IDRFs (15 single, five >1) were staged as INRG-L2. Complete and partial tumor resections were performed in 58/70 and 6/70 cases, while 6 had no resection. The overall complication rate was 17.1% (11/64). At a median follow-up of 9 years, five were lost to follow-up; 65 were alive. One patient with gross residue underwent total resection due to progression 13 years after the surgery, and one in the unresected group was lost to follow-up. Ten other cases without a complete resection experienced no tumor progression. CONCLUSIONS: Ganglioneuromas are benign PNTs, and most are free of IDRFs. Even without complete resection, long-term outcomes are excellent. Guidelines should be devised considering the high surgical complication rates and benign course of GNs. LEVEL OF EVIDENCE: Case series, IV.

Imaging Features of Pediatric Sarcoidosis.

Sarcoidosis is a granulomatous inflammatory disease of uncertain cause. It occurs most commonly in young and middle-aged adults and less frequently in children; therefore, few data on pediatric sarcoidosis exist in the literature. The diagnosis and management of sarcoidosis remain challenging because of diverse and often nonspecific clinical and imaging findings. In addition, the clinical picture varies widely by age. Prepubertal and adolescent patients often present with adult-like pulmonary disease; however, early-onset sarcoidosis is typically characterized by the triad of arthritis, uveitis, and skin rash. Sarcoidosis is mostly a diagnosis of exclusion made by demonstrating noncaseating granulomas at histopathologic examination in patients with compatible clinical and radiologic findings. Although sarcoidosis often affects the lungs and thoracic lymph nodes, it can involve almost any organ in the body. The most common radiologic manifestation is pulmonary involvement, characterized by mediastinal and bilateral symmetric hilar lymphadenopathies with perilymphatic micronodules. Abdominal involvement is also common in children and often manifests as hepatomegaly, splenomegaly, and abdominal lymph node enlargement. Although neurosarcoidosis and cardiac sarcoidosis are rare, imaging is essential to the diagnosis of central nervous system and cardiac involvement because of the risky biopsy procedure and its low diagnostic yield due to focal involvement. Being familiar with the spectrum of imaging findings of sarcoidosis may aid in appropriate diagnosis and management. ©RSNA, 2023 Test Your Knowledge questions are available in the supplemental material.

Liver magnetic resonance elastography and fat fraction in pediatric patients with cystic fibrosis versus healthy children.

BACKGROUND: Liver involvement is an important cause of morbidity and mortality in patients with cystic fibrosis (CF). While liver biopsy is the gold standard for demonstrating involvement, its invasiveness prompts a search for noninvasive alternatives. OBJECTIVE: To evaluate liver involvement in pediatric patients with CF (versus healthy controls) using magnetic resonance (MR) elastography/spectroscopy and to correlate the imaging findings with clinical/laboratory characteristics. MATERIALS AND METHODS: This was a single-center, prospective cross-sectional study conducted between April 2020 and March 2022 in patients with CF versus healthy controls. Patients with CF were divided into two subgroups: those with CF-related liver disease and those without. MR images were acquired on a 1.5-tesla machine. Kilopascal (kPa) values were derived from processing MR elastography images. MR spectroscopy was used to measure liver fat fraction, as an indication of hepatosteatosis. Groups were compared using either the Student's t test or the Mann‒Whitney U test. The chi-square test or Fisher's exact test were used to compare qualitative variables. RESULTS: Fifty-one patients with CF (12 ± 3.3 years, 32 boys) and 24 healthy volunteers (11.1 ± 2.4 years, 15 boys) were included in the study. Median liver stiffness (P=0.003) and fat fraction (P=0.03) were higher in the CF patients than in the controls. Median liver stiffness values were higher in CF patients with CF-related liver disease than in those without CF-related liver disease (P=0.002). Liver stiffness values of CF patients with high alanine aminotransferase (ALT), high gamma-glutamyl transferase, and thrombocytopenia were found to be higher than those without (P=0.004, P<0.001, P<0.001, respectively). Only the high ALT group showed a high fat fraction (P=0.002). CONCLUSIONS: Patients with CF had higher liver stiffness than the control group, and patients with CF-related liver disease had higher liver stiffness than both the CF patients without CF-related liver disease and the control group. Patients with CF had a higher fat fraction than the control group. Noninvasive assessment of liver involvement using MR elastography/spectroscopy can support the diagnosis of CF-related liver disease and the follow-up of patients with CF.

The risk of cardiovascular comorbidity in children with Behçet's disease.

OBJECTIVE: Patients with Behcet disease (BD) may experience long term morbidity caused by different forms of cardiovascular diseases. This study aimed to assess the risk for cardiovascular comorbidity in pediatric BD patients with and without vascular involvement, independent of the contribution of traditional risk factors. METHODS: Pediatric patients classified as BD according to the 2015 International Pediatric BD criteria were included in the study. Twenty-four-h ambulatory blood pressure monitoring (ABPM), transthoracic echocardiography, and carotid intima media thickness (cIMT) measurements were performed. Patients with an active disease and have other known risk factors for cardiovascular disease were not included in the study. RESULTS: Thirty-one children and adolescents with pediatric BD (16 female, 51.6%; F/M: 1.06) were enrolled in the study. Among all BD patients 10 patients (34.4%) had abnormal ABPM. Carotid IMT values, mean arterial pressure, systolic and diastolic blood pressure by ABPM and the prevalence of abnormal ABPM, non-dipping, and ambulatory hypertension were similar between patients with and without vascular involvement. The echocardiography measurements showed that BD patients with vascular involvement had significantly higher velocity and velocity time integral of the left ventricle outflow tract which may point out increased stiffness of the aorta. CONCLUSION: Pediatric BD patients with vascular involvement may tend to have more cardiovascular risk factors. However, cardiovascular assessment should be considered in all BD patients regardless of the involved systems. We suggest that ABPM may accurately define hypertension and cardiovascular risk in BD.

The role of distance between upper and lower esophageal pouches to predict the possibility of delayed repair in children with isolated esophageal atresia.

AIM: A retrospective study was performed to evaluate the role of distance between upper and lower esophageal pouches and pouch lengths to predict delayed primary repair (DPR) in patients with isolated esophageal atresia (EA). METHODS: Patients with isolated EA were evaluated for demographic features, associated anomalies, surgical options, and complications. The babygrams obtained for evaluating gap assessment were examined for the distance between pouches and pouch lengths. Patients were divided into two groups: DPR and esophageal replacement (ER). RESULTS: Fourteen cases with a mean age of 4.1 ± 1.9 years (1-9 years) were included. Female to male ratio was 6:8. There was no significant difference between DPR and ER groups for pouch lengths. The median distance between two pouches were significantly higher in ER group [50 mm (29.4-83.6) vs 18.8 mm (3.4-34.5) (p < 0.05)]. The distance between two pouches and pouch lengths were similar in patients with and without anastomotic strictures (p > 0.05). CONCLUSION: Both upper and lower pouch lengths were shorter in ER group compared to DPR group. However, pouch lengths cannot be considered as a single criterion to predict the need for ER. Large cohort of patients are needed to define the cut-off values for shortest pouch length to achieve a DPR.

Plastic bronchitis during childhood: Diversity of presentation, etiology, treatment, and outcomes.

OBJECTIVE: Plastic bronchitis (PB) is a rare disease in children, and reliable data are scarce. Here, we aimed to analyze the clinical features, management, and outcomes in children with PB. METHODS: The medical data of patients who were followed up with a diagnosis of PB between January 2010 and March 2022 were retrospectively analyzed. RESULTS: The median age of 15 patients was 9 (interquartile range: 4-10) years with a male/female ratio of 12/3. Initial symptoms included recurrent pneumonia (33.3%), persistent atelectasis (33.3%), cast expectoration (26.6%), and intense, persistent cough (6.6%). The most common underlying diagnosis was asthma (n = 12, 80%), and six of the patients were newly diagnosed. The most common radiological findings were atelectasis as a consequence of major airway obstruction on chest X-ray or computed tomography. Five patients, all diagnosed as having asthma, had recurrent PB and required multiple airway procedures for treatment and diagnosis. During a median 7-year follow-up of five patients, occasionally cast expectoration was observed in one patient with asthma who had poor compliance with inhaled corticosteroids. CONCLUSION: PB is a common reflection of the different underlying etiologies in the pediatric age group, and treatment and outcomes are closely related to these. It should be kept in mind that asthma can be a predisposing factor for the development of PB.

Endoscopic treatment of periampullary duodenal duplication cyts in an 18-month-old girl.

BACKGROUND: Duodenal duplication cysts (DDC) are rare congenital anomalies of the gastrointestinal tract and periampullary localization with anatomical variants including biliary and pancreatic duct anomalies remains a surgical challenge. Endoscopic treatment of the periampullary DDC (PDDC) communicating with the pancreaticobiliary duct in an 18-month-old girl is presented to discuss the endoscopic treatment options in children. CASE: An 18-month-old girl with a normal prenatal ultrasound (US) was asymptomatic until complaining of abdominal pain and vomiting at 10-months of age. Abdominal US revealed a 1.8 × 2 cm cystic mass adjacent to the second part of the duodenum. The amylase and lipase levels were slightly increased while she was symptomatic. Magnetic resonance cholangiopancreaticography (MRCP) showed a thick cyst wall measuring 1.5 × 2 cm at the second part of the duodenum, consistent with DDC that was suspected to be communicating with the common bile duct. Upper gastrointestinal endoscopy confirmed a bulging cyst in the duodenum lumen. The puncture and injection of the cyst with contrast material confirmed the communication of the duplication cyst with the common bile duct. The unroofing of the cyst was performed with endoscopic cautery. The biopsy obtained from the cystic mucosa revealed normal intestinal histology. Oral feeding was initiated six hours after the endoscopy. The patient has been followed for the last 8 months uneventfully. CONCLUSIONS: Endoscopic treatment of PDDC with various anatomical variants can be considered an alternative to surgical excision in children.

Acute Pancreatitis and Acute Recurrent Pancreatitis in Children: Imaging Findings and Outcomes.

OBJECTIVE: The prevalence of acute pancreatitis and acute recurrent pancreatitis in children has increased over the years, and there are limited data about imaging findings. This study aimed to reveal the imaging findings of acute pancreatitis and acute recurrent pancreatitis in children at a tertiary care hospital. MATERIALS AND METHODS: The patients with acute pancreatitis and acute recurrent pancreatitis diagnosed between January 2007 and December 2018 were included. Demographic and clinical features, follow-up period, and interventions were noted. Imaging features were evaluated for pancreatic enlargement, peripancreatic fluid, and biliary ducts for initial examination and pancreas parenchymal necrosis, peripancreatic collection, walled-off necrosis, pseudocyst, parenchymal atrophy, and biliary ductal dilatation for follow-up. RESULTS: The study included 74 patients with a mean age of 9 ± 4.9 years. The most common causes of acute pancreatitis and acute recurrent pancreatitis were biliary tract anomalies (n = 21), biliary ductal stones (n = 9), and cystic fibrosis (n = 8). Findings consistent with acute pancreatitis were determined by ultrasound in 40.5% (n = 30/74), whereas by magnetic resonance imaging in 60% (n = 39/65). Forty-one percent of the patients (n = 16) with positive magnetic resonance imaging findings did not show any findings on ultrasound. Acute recurrent pancreatitis was seen in 32 patients (43.2%). Follow-up imaging was performed in 55 patients (74.3%) between 2 months and 11 years. At follow-up, 8 patients had peripancreatic collections (6 walled-off necrosis and 2 pseudocysts). CONCLUSION: Recognizing the imaging findings of acute pancreatitis and its complications is crucial. Magnetic resonance imaging should be preferred as a second option following ultrasound, with the advantages of biliary ductal system delineation and better characterization of complications.

Are the thickness and ADC values reliable to evaluate pancreas in children? A retrospective MRI study.

PURPOSE: To define the pancreatic thickness on the transverse plane and apparent diffusion coefficient (ADC) values at multiple anatomical locations in the normal pancreas of children and to evaluate inter-rater and intra-rater reliability. METHODS: Two reviewers measured the thickness and ADC values of the pancreas at four locations obtained from two different 1,5 Tesla MRI scanners [MAGNETOM Symphony (Siemens Medical Systems, Erlangen, Germany) and Signa HDxt(GE Healthcare, Milwaukee, WI, USA)]. Measurements were made based on 190 retrospective MRI examinations. Patients were distributed among ten age groups for thickness measurements and three age groups for ADC measurements between 0 and 18 years. RESULTS: The thickness measurements from each segment of the pancreas were significantly correlated with patient age, height and weight (p < 0.01). Inter-rater agreement was excellent for pancreatic head (intraclass correlation coefficient (ICC): 0.837) and good for pancreatic neck, body and tail (ICC: 0.646, 0.632 and 0.678, respectively). We also defined the mean ADC values for three age groups. There was significant difference in the mean ADC value of the head and body by both the vendor and age group (p < 0.05). The mean ADC values and mean ranks of the Signa HDxt were significantly higher and the frequencies were lower than those of the MAGNETOM Symphony at all locations (p < 0.001). CONCLUSION: The thickness of the pancreatic head is more reliable than that at other locations. Pancreatic body and tail measurements are not as reliable as head or neck measurements due to discrepancies between published studies and low interobserver agreement. ADC values of the normal pancreas can be significantly different among vendors; therefore, ADC comparison on follow-up should be performed using same MRI machine.

Favourable outcomes of conservatively managed acute abdomen associated with multisystem inflammatory syndrome in children during the COVID-19 pandemic.

BACKGROUNDS: Abdominal pain is one of the most common symptoms of multisystem inflammatory syndrome in children (MIS-C). Abdominal pain can vary from mild to severe and may present as acute abdomen. Severe abdominal pain in patients with MIS-C should be differentiated from surgical causes of acute abdomen to prevent unnecessary surgery. METHODS: The diagnosis of MIS-C was based on WHO and CDC recommended criteria. Records of children treated for MIS-C between September 2020 and January 2021 were reviewed retrospectively. RESULTS: In a short time, we encountered seven patients who were diagnosed with MIS-C and showed acute abdomen findings. There were four male and three female patients. The median age was 9 years. Fever, abdominal pain and distension were present in all. The median duration of symptoms was 4 days. Five patients had general abdominal tenderness mimicking acute abdomen. Three patients had right lower quadrant tenderness mimicking acute appendicitis. After the initiation of immunomodulatory therapy and antibiotics, the physical examination findings were improved step by step in all. The median time to initiate oral feeding was 2 days. The median length of hospitalization time was 8 days. CONCLUSION: Serial abdominal examinations performed by the same surgeon enabled us to follow these patients conservatively and thus avoid unnecessary surgical intervention.

A new tool supporting the diagnosis of childhood-onset Behçet's disease: venous wall thickness.

OBJECTIVES: The lower extremity venous wall thickness (VWT) of Behçet's disease (BD) patients was reported to be significantly increased in adults, suggesting its use for the support of BD diagnosis. This prospective study aimed to investigate the lower extremity VWT in childhood-onset definite and incomplete BD patients and compare it to healthy age-matched controls. METHODS: Paediatric patients classified with BD according to the 2015 international paediatric BD criteria in our centre were included in the study. Intima-media thickness of the lower extremity veins to evaluate VWT was measured by ultrasonography, including common femoral vein (CFV), femoral vein (FV), vena saphena magna, vena saphena parva and popliteal vein (PV). RESULTS: In this cross-sectional study, VWT was measured in 35 patients (63% male) and 27 healthy controls (55% male). Thirteen (37%) of 35 patients met the criteria for the diagnosis of BD. The remaining 22 (63%) had incomplete BD and met two criteria. The median VWT values of both definite and incomplete BD patients were significantly higher than the control group in all veins on both sides. Regarding the best cut-off values of VWT for all lower extremity veins, the sensitivity rates were between 63% and 86%, while specificity rates were between 71% and 100%. CONCLUSION: Increased VWT was present not only in BD patients with vascular involvement but also in those without. We suggest that VWT may be a new criterion in supporting the diagnosis of childhood BD both in definite and incomplete BD patients.

Clinical Features and Outcomes of Gallbladder Polyps in Children.

BACKGROUND: Gallbladder polyps are rare lesions protruding into the gallbladder lumen with variable clinical presentation. No standard treatment algorithm has been developed for pediatric gallbladders, and the malignant potential of the gallbladder is not clear in children. Therefore, a retrospective study was performed to evaluate the clinical features and treatment options of gallbladder polyps in children. METHODS: Between 2014 and 2020, children diagnosed with gallbladder polyps were evaluated for age, gender, clinical features, results of follow-up with ultrasound findings, and treatment options retrospectively. RESULTS: The records of 15 patients with a mean age of 13.2 years (2-20 years) were included. The male: female ratio was 7 : 8. Gallbladder polyps was detected incidentally in 73.3% (n = 11) of the patients. Four (20%) of the patients were symptomatic (26.7%) and complained about abdominal pain. Laboratory tests were normal except in 3 patients who showed slightly increased liver function tests. Two of the patients had 3 polyps on admission. The polyps were 2-10 mm in size. The size of the polyp increased in 5 patients (33.3%) and disappeared in 4 patients (26.6%) in follow-up ultrasound examinations. Five of the patients underwent cholecystectomy and 1 of them was scheduled for surgery. Five of the asymptomatic patients who have polyps less than 10 mm in size are still on follow-up. In patients with cholecystectomy, the histopathology of gallbladders revealed cholesterol polyps (n = 2) and hyperplastic polyps (n = 2). One of the patients with cholecystectomy showed no polyps in histopathological evaluation. CONCLUSION: Despite the lack of a standardized algorithm, our data suggested that multiple polyps, polyps with increased in size or greater than 10 mm, and the presence of symptoms might require cholecystectomy in children. Asymptomatic patients with small-sized polyps can be identified using ultrasound, and the polyps may disappear during the subsequent follow-up.

Early postoperative complications in pediatric abdominal solid tumor surgery according to Clavian-Dindo classification.

PURPOSE: Postoperative period after abdominal solid tumor surgery is critical regarding complications. This study aimed to detect incidence and treatment of complications. METHODS: Single center retrospective study including years 2010-2019 for early postoperative complications were documented and graded according to Clavian-Dindo classification. RESULTS: The overall complication rate was 10% and they were chylous leak (n = 8, 2%), collection in surgical field (n = 8, 2%), acute renal failure (n = 7, 1.7%), hemorrhage (n = 5, 1.3%), intestinal obstruction (n = 4, 1%), surgical site infection (n = 3, 0.7%), thromboembolism (n = 3, 0.7%) and peripheric neuronal event (n = 2, 0.5%). Distribution of complications according to Clavian-Dindo classification was as follows: 2 grade I, 23 grade II, 9 grade IIIb, 5 grade IVa and 1 grade V. While age and percentage of benign or malignant disease were not different in groups with or without complications (p = 0.11, p = 0.24), males had more complications than female patients (p = 0.008). Having more than one surgery aiming tumor resection was associated with increased postoperative complications (p = 0.002). Incidence of complications were highest after extragonadal germ cell tumor (35%) and hepatic tumors (29%). CONCLUSIONS: Postoperative complications of abdominal tumor surgery are diverse in children. They are related with prolonged hospital stay and need for medical or surgical interventions. Number of surgeries, organ of origin and tumor type have an influence on risk of complications.

Childhood-onset Takayasu arteritis and immunodeficiency: case-based review.

Takayasu arteritis (TAK) has been rarely reported in patients with immunodeficiency. In this review, we present two cases with childhood-onset TAK (c-TAK) and primary immunodeficiency while reviewing similar cases in the literature. We reviewed the data for our two pediatric patients with c-TAK and primary immunodeficiency. We also reviewed the literature for patients with c-TAK and immunodeficiency from the inceptions of the databases up to November 2021. A 14-year-old patient had lipopolysaccharide-sensitive beige-like anchor (LRBA) deficiency, and a 16-year-old had X-linked severe combined immunodeficiency (X-linked SCID). During the follow-up, they developed findings suggestive of vasculitides such as hypertension, elevation in acute phase reactants, weakness, and weight loss. Thoracoabdominal computed tomography angiography revealed findings consistent with vasculitis involving the aorta and its major branches. Patients were diagnosed with c-TAK, and corticosteroids were given to both patients in the treatment. We identified 11 articles describing 17 TAK patients with immunodeficiency in our literature search. Two of the patients with c-TAK were infected with human immunodeficiency virus (HIV), another patient had Wiskott-Aldrich syndrome, and the other had idiopathic CD4 + T lymphocytopenia. Nine adult patients with TAK were infected with HIV, three patients had common variable immunodeficiency disorder (CVID), and the other had STAT1 gain-of-function mutation. Clinicians should consider that immunodeficiencies may be accompanied by vasculitic conditions such as TAK. Hypertension, increased inflammatory markers, and constitutional symptoms may be red flags for the development of TAK.