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Placental transmogrification of the lung (PTL) is a rare pulmonary condition characterized by the presence of immature placental villous structures. The etiology and molecular mechanisms underlying this disease remain largely unknown. This functional study aimed to identify the molecular signatures in the pathogenesis of PTL via comprehensive transcriptome analysis. Comparative transcriptomic assessment of PTL tissue and stromal cells showed differential expression of 257 genes in PTL tissue and 189 genes in stromal cells. Notably, several transcription factors and regulators, including FOSB, FOS, JUN, and ATF3, were upregulated in PTL tissue. Additionally, genes associated with the extracellular matrix and connective tissue, such as COL1A1, MMP2, and SPARC, were significantly altered, indicating possible fibrotic changes. Gene set enrichment analysis highlighted the role of vascular development and extracellular matrix organization, and the Activator Protein-1 (AP-1) transcription factor was significantly activated in PTL tissue. Furthermore, the analysis highlighted an overlap of 25 genes between PTL tissue and stromal cells, underscoring the importance of shared molecular pathways in the pathogenesis of PTL. Among the shared genes, JUND, COL4A2, COL6A2, IGFBP5, and IGFBP7 were consistently upregulated, highlighting the possible involvement of AP-1-mediated signaling and fibrotic changes in the pathogenesis of PTL. The present findings pave the way for further research into the molecular mechanisms underlying PTL and offer novel insights for therapeutic interventions. Given the rarity of PTL, these molecular findings represent a significant step forward in our understanding this enigmatic disease., Competing Interests: Declaration of Competing Interest There is no conflict of interest., (Copyright © 2024 Elsevier GmbH. All rights reserved.)

Competing Interests: The author declares that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

The testis develops in the abdominal cavity and descends into the scrotum. Although numerous theories have been proposed, the mechanism of descent and the reason for its inhibition remain unknown. Furthermore, none of the explanations account for the other occurrences related to the descent, such as failed obliteration of the processus vaginalis, or the reasons for the decrease in fertility and increase in the risk of malignancy associated with an undescended testis. The gubernaculum is a primitive mesenchymal tissue that was first described in 1786. However, the role of the gubernaculum in the descent process remains obscure. The testis descends through the processus vaginalis. Although the processus vaginalis (PV) is usually defined as a simple peritoneal protrusion, it actively develops into the gubernaculum. The gubernaculum gives rise to the smooth muscles that surround the processus vaginalis. The striated cremaster muscle (CM) is also derived from the gubernaculum. Because the testis descends through the processus vaginalis, the muscles develop to propel the testis. After propelling the testis, the smooth muscle (SM) undergoes programmed cell death. The initiation of programmed cell death through the intrinsic pathway requires activation of phospholipase C. A transient shift in the autonomic balance via a decrease in the sympathetic tonus and an increase in the parasympathetic tonus is essential for initiating this programmed cell death. Programmed cell death in the SM is the physiological pathway for the obliteration of the processus vaginalis. Differences in the timing, intensity, or duration of this physiological pathway result in pathological conditions. A shift before testicular descent diminishes the SM content that is required to propel the testis, and thus inhibits descent. The early shift persists throughout childhood and results in the decrease in fertility and increase in the risk of malignancy because of the differences in signal transduction. Despite a successful descent, persistence of the shift alters the contractility of the CM by increasing the cytosolic calcium levels. Contracted CMs retracts or even ascends the testis. Inadequate intensity or duration of the shift of autonomic tonus causes failure of the programmed cell death. Persistence of the SM hinders the obliteration of PV and gives rise to hydroceles or inguinal hernias depending on the amount of residual smooth muscles. Similar findings from different countries support these explanations. Thus, our proposed mechanism satisfactorily explains the process of descent while considering all the factors related to the process of testicular descent., Competing Interests: Conflict of Interest: No conflict of interest was declared by the author.

A retrospective study was performed to evaluate the role of oral feeding (OF) time and sham feeding (SF) on oral-pharyngeal swallowing functions in children with esophageal atresia (EA). Patients with EA were evaluated for age, sex, and types of atresia, time to start OF and surgical complications. Three to six weeks after full OF, videofluoroscopic swallowing evaluation (VFSE) was performed, and Penetration Aspiration Score (PAS, no aspiration = 1, penetration = 2-5, aspiration = 6-8), delay in swallowing reflex and residue after liquid and pudding consistency were noted. Patients were divided into three groups according to repair time; early primary repair (EPR, < 1 month of age), delayed primary repair (DPR, ≥ 1 month of age) and colonic interposition (CI). VFSE findings were compared between groups. In CI group, patients without aspiration in VFSE were encouraged to sham SF before CI. Patients with and without SF in CI group were also compared for oro-pharyngeal dysphagia (OPD). PAS scores were significantly higher in DPR (n = 13) group when compared to CI group (n = 12) in liquid swallowing (p = 0.032) and higher than EPR (n = 30) in pudding consistency (p = 0.03). Patients with CI showed significant OPD when compared to EPR (p = 0.017). Swallowing reflex delay was similar in both groups (p > 0.05). DPR group had significantly higher liquid residue at the level of vallecula (p = 0.028). The residue at other levels (oral, tongue base, pharyngeal wall and pyriform sinus) were similar in all groups in both liquid and pudding consistencies. There was no significant difference between sham-fed (n = 6) and not sham-fed (n = 6) infants in VFSE of OPD (p > 0.05). Patients with DPR without SF had significantly higher incidence of OPD and PAS scores when compared to EPR and CI. Although CI groups has the latest OF time, SF may improve oral motor abilities and cause better OF swallowing functions than patients with delayed repair., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Bullous lung diseases may cause primary spontaneous pneumothorax (PSP) in children. The microRNAs (miRNAs) are non-coding RNAs that participate in regulation of inflammation and cancer. We hypothesized that children with bullous lung disease and PSP may have altered miRNA expressions in their exhaled breath condensates (EBCs). Therefore, a prospective study was performed to evaluate the miRNA-24 and 21 expression, and the matrix metalloproteinase-7 (MMP-7) levels in EBC of children with PSP. Children with PSP were evaluated for age, gender, clinical features and results of surgical treatment. EBC samples (500-1000 ml) were collected to evaluate the miRNA-21, 24 expressions, and MMP-7, and tissue-inhibitor-MMP-1 (TIMP-1) levels. miRNA expressions and MMP levels of patients were compared with healthy controls (control group (CG), n = 12). Subjects ( n = 16) with a mean age of 15 years (10-19 years), and a male-to-female ratio of 14:2 were enrolled in this study. The most common presenting symptom was sudden chest pain ( n = 14). In 62.5% of the cases an underlying bullous lung disease were detected. During an average of 16.6 months (1-60 months) follow up period, four subjects relapsed. The mean MMP-7 (1.74-1.57 ng ml -1 ), and TIMP-1 (1.92-1.84 ng ml -1 ) levels were similar between both groups ( p > 0.05). miRNA-24 expression was significantly decreased in the PSP group, when compared to the CG (0.16-1 2 -ΔΔCT , p < 0.05). In addition, the miRNA-21 expression was not different between the two groups ( p > 0.05). In conclusion, the miRNA-24 levels were significantly decreased in children with PSP. Taken together, children with PSP, especially those with bullous disease, should be closely monitored in the long-term period., (© 2022 IOP Publishing Ltd.)

Purpose: Dysphagia is the most common symptom in patients with esophageal atresia (EA) of all ages. There is no study addressing the direct relation between dysphagia and surgical results. Therefore, a systematic review was performed to define the relationship between surgical complications and dysphagia in patients with EA., Methods: The systematic review was drafted under PRISMA guidelines. Systematic literature search was performed for the period 2000 (Jan) to 2019 (Dec)-20 years-in the databases: MEDLINE, EMBASE, and PubMed. Statistical analysis was performed using Comprehensive Meta-Analysis Version 3.0 software., Results: Among 767 articles, 486 abstracts were screened for the inclusion criteria. The full-texts of 64 articles were assessed for eligibility. The sub-group analysis could be performed in 4 articles for anastomotic strictures. Heterogeneity was calculated by I 2 statistic as 18,487 and pooled odds ratio was measured under the fixed effect model (Q = 3.68; P = 0.298, I 2  = 18,487). There was no significant relationship with an odds ratio of 1.37 between anastomotic stricture (AS) and dysphagia (95% CI 0.631-2.973, p = 0.426). There was no publication bias for the data (Begg's test, p = 0.496; Egger's tests, p = 0.335)., Conclusion: This meta-analysis did not reveal a significant relationship between AS and dysphagia in children with EA. Since many other factors contribute to dysphagia, comprehensive variable information such as detailed standardized registry systems for rare diseases for pooling analysis is needed regarding other potential factors including surgical complications., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To evaluate the management of children with severe gastrointestinal symptoms during the disease course of COVID-19 and multisystem inflammatory syndrome (MIS-C)., Methods: After ethical approval, we reviewed the medical records, retrospectively, of children with COVID-19 or MIS-C requiring surgical consultation for severe gastrointestinal symptoms., Results: The subjects comprised 15 children, 13 with MIS-C and 2 with COVID-19. Twelve children (80%) had been in known close contact with a person with SARS-CoV-19 and 13 were positive for Anti-SARS-CoV-2 IgG. All the children had experienced fever for at least 1 day and had signs of involvement of two or more systems. Three patients required surgical intervention: one underwent surgical exploration with a presumptive diagnosis of acute appendicitis in the referring center and was transported to our center following clinical deterioration, where a diagnosis of MIS-C was confirmed; and the remaining two developed appendicitis during hospitalization for COVID-19. All three patients had a longer duration of abdominal pain, a higher number of lymphocytes, and a lower level of inflammatory markers than the non-surgically managed patients. None of the patients presenting with MIS-C underwent surgical exploration., Conclusion: Gastrointestinal involvement may mimic acute abdomen in children with COVID-19. Thus, children presenting with acute abdomen in the pandemic era require careful evaluation and prompt diagnosis to avoid unnecessary surgical intervention., (© 2022. The Author(s) under exclusive licence to Springer Nature Singapore Pte Ltd.)

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.

Purpose: To investigate the occupational and environmental factors in the etiology of infantile hypertrophic pyloric stenosis (IHPS)., Methods: Protocol was drafted according to the PRISMA guidelines and registered on PROSPERO (CRD42020152460). A search for a combination of terms related to IHPS, fetus and neonates, and environmental exposure was performed for studies published between 2000 and 2020 in the EMBASE, Pubmed, and MEDLINE databases., Results: Overall, 2203 abstracts were identified and 829 were screened. The full text of the selected articles (N = 98) was assessed for eligibility. Fifteen studies were included in quantitative synthesis. IHPS risk was significantly lower in black and Hispanic mothers than in white mothers [OR 0.47 (95% CI 0.44-0.51, p < 0.001), OR 0.85 (95% CI 0.77-0.94, p = 0.002), respectively]. Lower maternal education level and maternal smoking were risk factor for IHPS. We further observed a non-significant association between maternal folic acid usage and IHPS risk. Data were insufficient to evaluate occupational exposure., Conclusion: This review provides an understanding of the role of environmental exposures in IHPS etiology. Lower maternal educational level, maternal smoking, and white ethnicity are associated with a significantly increased risk of IHPS, while folic acid use seems non-significantly associated with IHPS risk., Level of Evidence: III., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: This study was designed to evaluate the effectiveness of conservative treatment for chylous leak after tumor surgery and to propose a management algorithm., Methods: The data of patients with postoperative chylous leak after tumor surgery in our institution between 2010 and 2019 were retrospectively reviewed. In this study, 469 laparotomies, 89 thoracotomies, and 57 cervical excisions were performed for tumor surgery in our institution., Results: Twelve patients with a median age of 4 (IQR, 3-8) years had postoperative chylous leak. All patients received total parenteral nutrition for a median of 13 days. Five patients had intravenous somatostatin for a median of 14 days (IQR, 9-16) to decrease chyle production. Eventually, chylous leak ceased in all patients with conservative treatment and surgical drains were removed after no leak was observed with enteral feeding., Conclusions: The incidence of chylous leak in childhood tumor surgery is approximately 2%. Extended tumor resection and lymph node dissection lead to the injury of the delicate structures that drain chyle. Conservative treatment with total parenteral nutrition and somatostatin seems to be effective. In particular, somatostatin may be used in resistant cases. Conservative treatment can take up to 1 month. The algorithm consists of how to manage postoperative chylous leak in childhood., (© 2021. Springer Nature Singapore Pte Ltd.)

Background: To evaluate the predictability of clinical and radiological findings in the diagnosis of malrotation., Methods: Between 2010 and 2020, children with presumptive diagnosis of malrotation were included. The demographic features, clinical and radiological findings, operative findings and outcome were recorded. The upper gastrointestinal series (UGIS) were evaluated by two radiologists. All parameters were correlated with surgical findings to evaluate the predictability., Results: Seventy patients were included. The presenting symptom was bilious vomiting in 29 cases (41.4%), and atypical symptoms (non-bilious vomiting, food refusal, etc.) in 40 cases (57%). One of the cases (1.6%) was asymptomatic and diagnosed incidentally during UGIS. 52 cases had abdominal X-ray and 14 (26.9%) of them were normal. Doppler ultrasonography (US) (n=20) revealed evidence of malrotation in 13 cases (65%). The location of duodenojejunal junction (DJJ) in UGIS was compatible with malrotation in 33 cases. 48 (61%) cases underwent surgical exploration; 35 cases had malrotation and seven cases had midgut volvulus. Median followup time was one year (0.5-7 years). Volvulus has recurred in one case and another case operated for volvulus died because of short bowel syndrome. The statistical analysis for predictability revealed that bilious vomiting (sensitivity: 57.1%, specificity: 82.1%), Doppler US (sensitivity: 92.3%, specificity: 75%) and right-sided DJJ in UGIS (sensitivity: 96.8%, specificity: 75%) have highest predictability., Conclusions: The bilious vomiting, Doppler US findings and right-sided DJJ have the highest predictability to confirm the diagnosis. However, presenting with atypical symptoms and having atypical or normal findings in UGIS do not rule out malrotation.

Aim: Pheochromocytoma (PCC) and paraganglioma (PGL) are rare tumors in childhood. They are catecholamine secreting tumors and present with signs or symptoms related to their excess. Most common signs and symptoms are hypertension, headache and diaphoresis. The management of children usually depend on experience of adulthood. This study is conducted to present the clinical characteristics, surgical management and outcome of childhood PCC and PGL in a tertiary care center., Material and Methods: We reviewed clinical records of all patients operated for PCC and PGL between 2000 and 2020 retrospectively., Results: There were 18 children operated for PCC and PGL in the study period. The female to male ratio was 1:1. The median age at diagnosis was 13 (IQR, 9-15) years. The most common presenting symptoms were headache and diaphoresis. Hypertension was the most common sign. Three patients had von Hippel-Lindau (VHL). Tumors of two patients with VHL were detected during routine follow-up. Three patients had multifocal disease. Medical preparation for surgery was carried out in all patients. Antihypertensive treatments were administered preoperatively. Since the patients are at risk for postoperative hypotension due to chronic vasoconstriction and blood volume contraction, high salt diet was recommended. Intravenous normal saline at a rate of 3000 ml/m 2 body surface area per day was started for intravascular volume expansion preoperatively. The mean duration for preoperative medication to achieve normal blood pressure was 22 days (range, 16-30). Twenty-five tumors were excised in eighteen patients. One patient who had bone metastases on diagnosis and is on I 131 MIBG therapy. The median follow-up time was 5.6 years (range, 1 months - 21 years). Five patients reached adulthood during the study period. Four of these had recurrent metastases (n = 2) and new tumors (pancreatic neuroendocrine tumor, n = 1 and pancreatic neuroendocrine tumor and renal cell carcinoma, n = 1) after the age of 18., Conclusion: Multidisciplinary approach is necessary to achieve safe surgical treatment and surveillance of PCC and PGL. Detection of associated familial cancer susceptibility syndromes and long-term follow-up is essential to detect late recurrences and new tumors., Competing Interests: Conflicts of interest We have no conflicts of interest to declare., (Copyright © 2021 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.)

Background: Adrenocortical tumours (ACT) are rare tumours of childhood usually presenting with endocrine dysfunction. This retrospective study is designed to review our institutional experience in surgical management., Methods: Records of children treated for ACT between 1999 and 2019 were reviewed retrospectively., Results: The median age of 24 children was 78 months. Fourteen patients had adrenocortical carcinoma, nine had adrenocortical adenoma and one had neuroendocrine differentiation of ACT. Endocrine dysfunction was noted in 79% of the patients. Five patients had preoperative chemotherapy but none had a decrease in tumour size. Transabdominal approach was used in all but two patients who had thoracoabdominal incision for excision of giant tumours and ipsilateral lung metastases. Two patients had visceral excision to achieve R0 resection. Five patients, four of whom had spillage and one with partial resection died of widespread disease. Two patients with stage 4 adrenocortical carcinoma are still on chemotherapy. All patients with stage I-III disease who had total excision without spillage (n = 17) are disease-free for 2-170 months., Conclusions: Our results show the importance of excision in ACT without spillage for survival. However, multicentre prospective studies should enhance the knowledge of children about ACT and develop alternative therapies for stage III and IV cases., (© 2021 Royal Australasian College of Surgeons.)

MicroRNAs (miRNAs) are noncoding RNAs that play an important role in the regulation of inflammation and have not been evaluated in exhaled breath condensates (EBC) of patients with esophageal atresia and tracheoesophageal fistula (EA-TEF). It is aimed to evaluate the levels of miRNA-21 and miRNA-24 in EBC of patients with EA-TEF. Patients who received surgery for EA-TEF (EA) were assessed for age, sex, types of anomaly, surgical treatments, and respiratory problems. A 500-1000 mL of EBC was obtained from each participant with EcoScreen. The levels of miRNA-21 and miRNA-24 in the EBC were analyzed by real-time polymerase chain reaction and compared between the EA group and the control group consisting of healthy children with no history of respiratory problems (n = 17). The levels of miRNAs in relation to respiratory problems and gastroesophageal reflux (GER) were also assessed. A total of 19 patients were enrolled in the EA group with a mean age of 7.8 ± 3.2 years and a male-to-female ratio of 10:9 EA cases had significantly lower levels of miRNA-21 (P < 0.05) compared to that in control group. The miRNA-24 levels did not differ between groups (P > 0.05). EA patients with positive pH testing for GER (n = 6) and fundoplication (n = 6) had higher levels of miRNA-21 than those with normal pH testing and without fundoplication, respectively (n = 13, P < 0.05). The levels of miRNA-21 and miRNA-24 did not differ between patients with and without proton pump inhibitor treatment (P > 0.05). The lower levels of miRNA-21 in the EBC of EA patients suggest a hyperreactive airway problem, which may be associated with GER and its surgical treatment., (© The Author(s) 2020. Published by Oxford University Press on behalf of International Society for Diseases of the Esophagus. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Infantile hypertrophic pyloric stenosis (IHPS) is one of the hallmark pediatric surgical diseases. However, its etiology remains incompletely understood. By systematically reviewing the literature, we aim to clarify the effect of the effect of occupational and environmental factors and role of nitric oxide (NO) metabolism in the etiopathogenesis of IHPS. The systematic review is drafted with the Preferred Reporting Items for Systematic Reviews and Meta-Analysis statement (PRISMA) and the Meta-analysis of Observational Studies in Epidemiology (MOOSE). Systematic literature search will be performed for the period 2000 (Jan) to 2020 (Dec) in the databases: MEDLINE, EMBASE, PubMed. The systematic search will cover the literature in English and Turkish language and will be limited to studies on human subjects. Four investigators will independently search the databases (MEDLINE, EMBASE, PubMed) according to the defined search strategy. The full-text of the selected articles will be screened independently by four reviewers, against the inclusion criteria. Descriptive data will be extracted from each study regarding: study details, methods, participants, outcomes and calculations of association for potential further statistical analysis. If meta-analysis could not be undertaken, systematic approach to analyzing the findings of included multiple studies will be described. Heterogeneity will be assessed by quantifying the inconsistency across studies using I2 statistic. Statistical analysis will be performed using Comprehensive Meta-Analysis Version 3.0 software. The p values lower than 0.05 will be considered statistically significant for all analyses., Competing Interests: The authors have declared that no competing interests exist.

Purpose: To propose a new scoring system to predict foreign body aspiration (FBA) in children., Methods: Children who underwent bronchoscopy for FBA were evaluated for age, sex, history of aspiration, physical examination, radiological findings and results of bronchoscopy retrospectively. A new proposed FBA scoring including statements about history, physical and radiological findings were applied to all patients to define a total score. The results of each statement and total FBA score were compared according to bronchoscopy findings. The sensitivity and specificity of FBA score and cut-off values of total FBA score to predict positive cases were evaluated., Results: Totally 720 patients with a male to female ratio of 227:133 were included. FBA was noted in 52.1% (n=375) of cases. When the scoring system compared with the existence of foreign body (FB), the patient history had no statistical significance to predict positive cases (p>0.05). The existence of FB was significantly associated with physical examination, radiological findings and total FBA score (p<0.001). The revised scoring system without history parameters had range of total scores 0 to 5. The total scores were obtained by sum of physical findings and radiological parameters and the cut-off value of 2 had the highest diagnostic performance with sensitivity and specificity of 77.9% and 74.8% to predict FBA in children., Conclusion: Our results suggest that the proposed scoring system can be utilized to predict FBA in children. The total score higher than 2 is predictive for FBA. Although, physical and radiologic findings are strong parameters for positive bronchoscopy, the history of FBA has no diagnostic utility., Level of Evidence: Level III (retrospective comparative study) STUDY TYPE: Diagnostic study., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background/aims: Pediatric intestinal pseudo-obstruction (PIPO) is the most severe form of intestinal dysmotility in children. This study aims to present the cases of PIPO to discuss its diagnosis, management, and prognosis., Materials and Methods: We retrospectively analyzed the medical records of the patients with PIPO between 2010 and 2018., Results: A total of 7 patients were included. The admission age was 3 days-10 years. The complaints were abdominal distention and constipation in all the patients. All the patients had passed meconium in the first 48 hours of their life. An upper gastrointestinal (GI) series revealed slow transit in 6 patients and malrotation in 2 patients. Full-thickness rectum biopsies revealed normal ganglion cells. Neurological examination revealed postinfectious pandysautonomy in 1 patient. Furthermore, 2 patients are under follow-up with ileostomy and TPN, 1 patient is with enteral feeding and ileostomy, and 3 patients are stable with pyridostigmine, enemas. Moreover, 1 patient died because of sepsis. The prognosis was not significantly correlated with initial presentation time, lag time, and presence of extraintestinal manifestations (p>0.05). The prognosis was significantly better when fewer number of operations were performed (p=0.029) Conclusion: PIPO is a broad-spectrum disease group that is difficult to diagnose and treat. It is mandatory to rule out the secondary causes of diagnosis. Medical and surgical treatments are used to support the nutritional status, prevent sepsis, and restore the intestinal motility. The prognosis was better when the secondary causes were identified and fewer operations were performed.

Aim: To evaluate the relationship between respiratory problems and oxidative stress markers in exhaled breath condensate (EBC) of patients with esophageal atresia (EA)., Methods: EA cases with respiratory problems were evaluated retrospectively for age, gender, the type of atresia, surgical treatment, outcome and respiratory symptoms. The results of gastroesophageal reflux (GER) treatment including the use of proton pump inhibitor (PPI) and fundoplication were also documented. EBC samples of 500-1000 μl were obtained by Ecoscreen machine in all cases. The levels of Glutathione (Glut), 8-isoprostane (8-iso), cysteinyl-leukotriene (Cys-LT) were measured with ELISA. Results were compared with healthy control subjects (CG, n = 26) and the relationship between oxidative stress markers and respiratory symptoms was evaluated. The results of GER treatment and oxidative stress markers in EBC were also correlated., Results: Twenty-nine patients with a mean age of 8.8 years (3-14 years) were included. The male/female ratio was 16:13. The EA presented with distal fistula in 27 cases. While no fistula was observed in 1 case, both proximal and distal fistulae were present in another single case. Associated anomalies, most of which were cardiovascular anomalies, were observed in 65.5% (n = 19) of cases. The median Glut level was 1.03 mM/ml (0.93-1.15), iso-8 was 38.8 pg/mL (32.03-76.2) and Cys-LT was 0.44 pg/mL (20.17-61.3) in patients with EA. The median levels of oxidative markers in CG were 1.23 mM/mL (1.13-1.36), 66.3 pg/mL (33.5-106.7), and 56.9 pg/mL (27.4-80.1), respectively. Glut levels were significantly lower in EA cases compared to CG (p = 0.01). There was no significant difference between the groups regarding 8-iso and CYS-LT levels (p = 0.9, p = 1.0). Cys-LT levels were significantly lower in patients with PPI treatment [21.7 pg/mL (18.6-48.1)], when compared to patients without PPI treatment [41.1 pg/mL (22.5-83.1)] (p = 0.04) and healthy subjects [56.9 pg/mL (27.4-80.1)] (p = 0.017). The 8-iso levels were significantly decreased in cases with fundoplication compared to the patients without fundoplication (p = 0.02)., Conclusion: Glut - an antioxidant agent - levels were significantly lower in EBC of EA cases. The decrease in Cys-LT levels in cases with PPI treatment and in 8-iso levels in patients with fundoplication suggests that the oxidative damage in EBC of EA cases may be correlated with GER and its management., Type of Study: Case control study LEVEL OF EVIDENCE: Level III., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Aim: Primary spontaneous pneumothorax (PSP) is a rare pulmonary pathology that occurs in the absence of known lung disease. A retrospective study was performed to evaluate the results and outcome of PSP treatment in adolescents., Methods: The cases with PSP from January 2004 to December 2017 were evaluated for age, sex, family and smoking history, clinical and radiological findings and results of treatment., Results: Ten cases with PSP were included. The mean age of the patients was 15 years (10-17 years) and the male to female ratio was 9:1. Two of the patients (20%) had family history of PSP and four cases (40%) had smoking history. The initial complaints were chest pain (n=8), acute onset of cough (n=1) and breathing difficulty (n=1). Tube thoracostomy was performed in nine cases in which three of them were bilateral. Chest computed tomography (CT) demonstrated bullae (n=4; 40%) and subpleural blebs (n=2; 20%). Pleurodesis with talc was performed in four patients with pneumothorax for longer than a week in follow-up (n=4; 40%). Five cases had recurrent PSP within one year (n=5; 50%) and underwent pleurodesis with talc (n=4), autologous-blood (n=1) and bleomycin (n=1). Bleb excision was performed in two cases with persistent pneumothorax despite pleurodesis., Conclusion: Tube thoracostomy and oxygen supplementation, are considered as initial and adequate treatment of PSP in most of the adolescents. Prolonged air leaks require pleurosdesis as the first line treatment and surgical excision of blebs should be reversed for the patients who are unresponsive to other treatment options., (© 2020 Walter de Gruyter GmbH, Berlin/Boston.)

The study aimed to assess concerns of caregivers of children with EA-TEF related to feeding-swallowing difficulties, compare the concerns according to type of atresia and repair time, and investigate its relationship with time to start oral feeding. Caregivers accompanying 64 children with EA-TEF were included. Age, sex, type of atresia, repair time, and time to start oral feeding were noted. Parents completed the Turkish version of the Feeding/Swallowing Impact Survey (T-FS-IS) to assess the concerns of caregivers related to feeding-swallowing difficulties. The T-FS-IS has three subscales including daily activities, worry, and feeding difficulties. The median age of patients was 3 (min = 1, max = 12) years, of which 57.8% were male. 43.8% of cases were isolated-EA, and 56.3% were EA-distal TEF. 57.8% of cases received early repair, and 42.2% had delayed repair. The median time to start oral feeding was 4 weeks (min = 1, max = 128). The mean scores of daily activities, worry, feeding difficulties, and total score from the T-FS-IS were 2.43 ± 1.18, 2.73 ± 1.28, 2.10 ± 0.97, and 2.44 ± 1.09, respectively. Caregivers of children with isolated-EA reported more problems in total score and all subscales of the T-FS-IS than EA-distal TEF (p < 0.01). Caregivers of children who received delayed repair reported more problems in total score and all subscales of the T-FS-IS than children with early repair (p < 0.05). Moderate to strong correlations were found between the T-FS-IS and time to start oral feeding (p < 0.01, r = 0.55-0.65). This study suggests that caregivers of children with isolated-EA and/or delayed repair and/or delay in oral intake may have higher concerns related to feeding-swallowing difficulties.

Background/purpose: Chewing disorders (CD) may cause restrictions in solid food intake and can be seen in 37% of children with esophageal atresia-tracheoesophageal fistula (EA-TEF). The Functional Chewing Training (FCT) is a holistic approach to improve chewing function (CF) in children. The study aimed to evaluate the effects of FCT on CF in children with EA-TEF., Methods: Twenty children with CD were included. Patients received 12 weeks FCT including impairment-based and adaptive components. Chewing performance level was scored with Karaduman Chewing Performance Scale (KCPS), and tolerated food texture was determined by the International Dysphagia Diet Standardization Initiative (IDDSI). The baseline and final levels of KCPS and IDDSI were compared to evaluate the effects of FCT on CF., Results: Forty-five percent of cases were isolated-EA, and 55% were EA-distal TEF with a median age of 31 (min = 25, max = 84) months, of which 65% (n = 13) were male. Baseline evaluation showed that 12 cases were in level-1, 6 cases in level-3 and 2 cases in level-4 according to KCPS. Eight children with CD (40%) had IDDSI level-3 and 12 (60%) had level-7. There was a significant improvement in KCPS scores and IDDSI scores after 12 weeks of training (p < 0.01, p = 0.005,respectively). KCPS scores showed level-0 in 15 cases, and level-1 in 5 cases. All children had IDSSI level-7., Conclusions: The FCT is an effective method to improve chewing function in children EA-TEF who had CD., Type of Study: Single group treatment study., Level of Evidence: Level 4., Competing Interests: Conflicts of interest None., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Recurrence of tracheoesophageal fistula is a frequent complication after esophageal atresia repair. Acquired tracheoesophageal fistulas are long new fistulas that are localized at sites that are not typical of the congenital tracheoesophageal fistula. We present four cases to discuss the diagnostic and management challenges concerning various acquired tracheoesophageal fistula localizations., Case Report: We retrospectively evaluated the medical records of patients admitted with acquired tracheoesophageal fistula in the last 5 years. Among the 16 postoperative tracheoesophageal fistulas, 4 were classified as acquired tracheoesophageal fistula. Patients’ admission age ranged from 1 to 8 years. The female to male ratio was 2:2. The presented cases were admitted with recurrent respiratory tract infections, choking, and coughing. The acquired tracheoesophageal fistulas were observed between the esophagus and cervical trachea, between the esophagus and the right bronchus passing through intrathoracic abscess cavity, in the right bronchus, and between the colon conduit and trachea. One of the acquired tracheoesophageal fistulas healed spontaneously, whereas others required surgical ligation., Conclusion: Acquired tracheoesophageal fistula most often occurs secondary to local or diffuse mediastinitis. Acquired tracheoesophageal fistula may appear at unusual sites not typical of congenital tracheoesophageal fistula, such as esophagus-to-right bronchus and conduit to trachea. Therefore, the unusual locations of acquired tracheoesophageal fistula should be borne in mind, and patients evaluated and managed more comprehensively.

Background: Foreign body aspiration (FBA) is a serious life-threatening condition in childhood. "Baby-led weaning (BLW)" is a popular method in which the babies are encouraged to self-feed to gain oral motor abilities. The role of BLW in FBA is controversial. A retrospective study was performed to evaluate the results of FBA in infants (<1 year of age) and its relation to the feeding method. Materials and Methods: Children who underwent bronchoscopy for FBA for the past 10 years were included. Infants (<1 year of age) were evaluated for age, gender, clinical findings, and the results of bronchoscopy. The type of feeding, including self-feeding or caregiver-assisted feeding, was noted. Results: The medical records of 826 patients who underwent bronchoscopy were evaluated. FBA was noted in 50.2% ( n  = 417) of cases. Only 9.07% ( n  = 75) of patients were <1 year of age and 67% ( n  = 50) of them had a foreign body according to the bronchoscopy. The mean age was 9 months (5-12 months) and 36% of them were male. When the feeding characteristics of patients were surveyed, 80% of cases aspirated when self-feeding and 14% aspirated during caregiver-assisted feeding. Conclusions: Self-feeding to promote oral motor function may cause FBA in infants. Emergent bronchoscopy is more common in infants and reveals the aspiration of foods that cannot be consumed safely in this age group., Competing Interests: No competing financial interests exist.

Background/aims: The present study aimed at investigating the long-term outcomes and prognostic factors of patients with biliary atresia (BA) diagnosed and followed at a single center., Materials and Methods: Patients with BA treated during 1994-2014 at a large-volume pediatric tertiary referral center were reviewed retrospectively with regard to demographic, clinical, laboratory, and diagnostic characteristics for identifying the prognostic factors and long-term clinical outcomes., Results: Overall, 81 patients (49 males, 32 females) were included. Mean age at diagnosis was 73.1±4.7 (median: 64) days. Of the patients included, 78 patients (96%) underwent a portoenterostomy procedure. Mean age at operation was 76.8±4.7 (median: 72) days. The surgical success rate was 64.8%. A younger age (either at diagnosis or surgery) was the only determinant of surgical success. The 2-, 5-, and 10-year overall survival (OS) rates, including all patients with or without liver transplantation, were 75%, 73%, and 71% respectively, whereas the 2-, 5-, and 10-year survival rates with native liver (SNL) were 69%, 61%, and 57%, respectively. Mean follow-up duration was 9.4±7.5 years. Successful surgery, presence of fibrosis and/or cirrhosis on the liver pathology, and prothrombin time [international normalized ratio (INR)] at presentation were independent prognostic factors for both OS and SNL., Conclusion: A younger age at diagnosis is strongly associated with surgical success in BA. Surgical success, the prothrombin time (INR) at presentation, and liver pathology are independent prognostic factors affecting the long-term outcomes in patients with BA. Therefore, timely diagnosis and early referral to experienced surgical centers are crucial for optimal management and favorable long-term results in BA.

Introduction:  Feeding problems are common in children with esophageal atresia and tracheoesophageal fistula (EA-TEF); however, chewing disorders, which may cause inability to intake solid food, have not been evaluated. Therefore, we aimed to evaluate the chewing function in children with repaired EA-TEF., Materials and Methods:  Age, sex, the type of atresia, the type of repair, and the time to start oral feeding were recorded. The level of the chewing performance was scored according to the Karaduman Chewing Performance Scale (KCPS). The International Dysphagia Diet Standardization Initiative (IDDSI) was used to determine the tolerated food texture in children., Results:  A group of 30 patients were included, of which 53.3% was male. The percentages of the isolated-EA and that of the EA-distal TEF were 40% and 60%, respectively. The median value for the time to start oral feeding was 4.5 weeks (min = 1, max = 72). Eleven (36.7%) children had chewing disorder. The KCPS scores showed level I in six cases, level III in four cases, and level IV in one case. Five children with chewing disorder had IDDSI level 3 and six had level 7, along with the sensation of stuck food. We found no significant difference between the KCPS scores according to the repair type ( p  = 0.07). The median values of the KCPS scores of children with primary repair, delayed repair, and colon interposition were 0 (min = 0, max = 4), 0.5 (min = 0, max = 3), 2 (min = 0, max = 3), respectively. A significant positive correlation was found between the time to start oral feeding and the KCPS scores ( r  = 0.63, p  = 0.001)., Conclusion:  Chewing disorders can be observed in children with EA-TEF, and the type of repair and the delay in oral feeding may be related to chewing disorder. Therapeutic maneuvers are needed to improve the chewing function in children with EA-TEF., Competing Interests: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Introduction: Neuroendocrine tumors (NET) within tailgut cysts (TC) are usually seen in middle-aged females. A 14 year-old boy who underwent surgical excision of NET within TC is presented to discuss the clinical features, diagnosis and treatment options., Case Report: A 14-year-old boy with rectal bleeding and constipation had a 5 × 4 cm mass detected upon rectal examination. The cystic mass was totally excised. The histopathological evaluation revealed a low-grade NET within mucularis layer of the cyst showing a positive immunhistochemical staining for cytokeratin and synaptophysin., Conclusion: Although, the malignant transition of TC is commonly observed in middle age females, males and children can also be affected. This is first report of a pediatric TC with malignant transformation. Total surgical excision of cysts is mandatory for local control of the disease and chemotherapy should be reserved for the patients who have positive surgical margins.

Aim  The aim of this study was to evaluate the results of Swallowing Rehabilitation Protocol (SRP) on swallowing function (SF) of esophageal atresia and tracheoesophageal fistula (EA-TEF) patients with pharyngeal swallowing disorder. Materials and Methods  In this study, 24 children with EA-TEF who had deglutitive and respiratory problems were grouped into either study ( n  = 12) or control group ( n  = 12) by basic randomization. Study group received the SRP including neuromuscular electrical stimulation, thermal tactile stimulation, and hyolaryngeal mobilization. The control group received nonnutritive stimulations. SF was evaluated with 3 mL liquid and pudding barium by videofluoroscopic swallowing study (VFSS) before and after 20 sessions of interventions. Results  No statistical differences were found between groups in terms of descriptive characteristics including age, sex, weight, height, type of atresia, repair type, repair time, and start time of oral intake ( p  > 0.05). There were no statistical differences between groups in term of swallowing parameters ( p  > 0.05), except reflux ( p  = 0.004) according to VFSS findings. After 20 sessions of interventions, the study group showed improvement in penetration-aspiration scale scores, oral phase dysfunction, delay in swallowing reflex, and residue in valleculae and pyriform sinuses after pudding swallow. The control group showed improvement only in oral phase dysfunction. Conclusion  SRP can be recommended to improve SF in patients with EA/TEF who have pharyngeal swallowing disorders., Competing Interests: Conflict of Interest: None., (Georg Thieme Verlag KG Stuttgart · New York.)

Aim: Airway aspiration is a common problem in children with esophageal atresia (EA). Pediatric Eating Assessment Tool-10 (pEAT-10) is a self-administered questionnaire to evaluate dysphagia symptoms in children. A prospective study was performed to evaluate the validity of pEAT-10 to predict aspiration in children with EA., Methods: Patients with EA were evaluated for age, sex, type of atresia, presence of associated anomalies, type of esophageal repair, time of definitive treatment, and the beginning of oral feeding. Penetration-aspiration score (PAS) was evaluated with videofluoroscopy (VFS) and parents were surveyed for pEAT-10, dysphagia score (DS) and functional oral intake scale (FOIS). PAS scores greater than 7 were considered as risk of aspiration. EAT-10 values greater than 3 were assessed as abnormal. Higher DS scores shows dysphagia whereas higher FOIS shows better feeding abilities., Results: Forty patients were included. Children with PAS greater than 7 were assessed as PAS+ group, and scores less than 7 were constituted as PAS- group. Demographic features and results of surgical treatments showed no difference between groups (p>0.05). The median values of PAS, pEAT-10 and DS scores were significantly higher in PAS+ group when compared to PAS- group (p<0.05). The sensitivity and specificity of pEAT-10 to predict aspiration were 88% and 77%, and the positive and negative predictive values were 22% and 11%, respectively. Type-C cases had better pEAT-10 and FOIS scores with respect to type-A cases, and both scores were statistically more reliable in primary repair than delayed repair (p<0.05). Among the postoperative complications, only leakage had impact on DS, pEAT-10, PAS and FOIS scores (p<0.05)., Conclusions: The pEAT-10 is a valid, simple and reliable tool to predict aspiration in children. Patients with higher pEAT-10 scores should undergo detailed evaluation of deglutitive functions and assessment of risks of aspiration to improve safer feeding strategies., Level of Evidence: Level II (Development of diagnostic criteria in a consecutive series of patients and a universally applied "gold standard")., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Aim: A prospective study was performed to evaluate anatomical alterations and hyolaryngeal elevation (HE) by videofluoroscopic swallowing study (VFSS) in patients with esophageal atresia-tracheoesophageal fistula (EA-TEF)., Methods: Patients operated for EA-TEF were evaluated for age, sex, type of atresia and time to esophageal anastomosis. All patients were evaluated by videofluoroscopic swallowing study (VFSS). Penetration-Aspiration scale (PAS≥7 is considered as aspiration), distance between upper esophageal sphincter and 2nd cervical vertebrae (UES-C2) and hyolaryngeal elevation (HE) were evaluated by the same deglutitionist who was blind to the study. The results of EA-TEF patients were compared with healthy children., Results: Eighteen patients with EA-TEF and 10 healthy controls were included. The median age was 16months (12-36m) in EA-TEF and 18months (13-51m) in controls. Male-to-female ratio was 5:4 and 4:1 respectively. 12 of cases were isolated-EA, 1 of them was EA-proximal TEF and 5 of the cases were EA-distal TEF. Half of the cases had primary EAN and others underwent delayed esophageal repair. Early oral feeding was also started in 9 patients (50%) whereas others had delayed oral feeding. VFSS showed aspiration in 27.7 (n=5) of cases (PAS≥7) in EA group. The median distance between UES-C2 was 3.04cm (min: 2.17-max: 3.94) in EA and 4.17cm (min: 3.45-max: 6.24cm) in controls. Median distance for HE was 0.37cm (min: 0.18-max: 1.1cm) in EA and 1.15 (min: 0.61-max: 1.06cm) in controls. The distance between UES-C2 was significantly lower than controls (p<0.05) and HE was decreased in EA-TEF without any statistical significance., Conclusion: Children with EA-TEF had shortened distance between airway and upper esophagus. HE may be inefficient to protect airway during deglutition. Anatomical alterations after EAN suggest that airway problems may be related with decreased HE in children with EATEF., Level of Evidence: Level II (Development of diagnostic criteria in a consecutive series of patients and a universally applied "gold standard")., (Copyright © 2017 Elsevier Inc. All rights reserved.)