Search

Background Malnutrition increases the complications and mortality in critically-ill children. A retrospective study was performed to define the role malnutrition on outcomes of multisystem inflammatory syndrome (MIS-C) due to COVID-19 in children. Methods Patients with MIS-C due to COVID-19 were evaluated for demographic features, anthropometric parameters, clinical findings and outcomes. Patients with z scores of body mass index (> 5 years-of-age) and weight-for-age (< 5 years-of-age) < -2 were considered as malnourished. Sarcopenia was defined by total psoas muscle area (tPMA), calculated on abdominal computed tomography (CT) at the level of L3 and L4 vertebrae. The z scores 0.05). Conclusion Children with MIS-C due to COVID-19 already have mild to severe malnutrition at admission. Rash and higher ferritin levels are more common in patients with malnutrition. In addition to anthropometric parameters, sarcopenia calculated using tPMA can be used to predict malnutrition in critically-ill children.

Background This article evaluates the effect of coronavirus disease 2019 (COVID-19) pandemic on clinical course and management of cases that underwent bronchoscopy for suspected foreign body aspiration (FBA) in children. Method The patients who underwent bronchoscopy with a presumptive diagnosis of FBA between July 2018 and December 2021 were evaluated for demographic features, clinical findings, management details, and outcomes. Patients were divided in two groups: before pandemic (group A) and during pandemic (group B). Results In total 79 cases with a median age of 5 years (4–5) in group A (n = 47) and 3 years (2–3) in group B (n = 32) were included (p Conclusion During pandemics, bronchoscopy for FBA was performed in younger infants than before pandemic and witnessed aspiration was significantly more common in that period. The differences in age groups and symptoms may be explained by spending more time at home during pandemics. Waiting for the PCR test results causes delays in the intervention. However, this delay did not cause any respiratory distress.

To evaluate the management of children with severe gastrointestinal symptoms during the disease course of COVID-19 and multisystem inflammatory syndrome (MIS-C).After ethical approval, we reviewed the medical records, retrospectively, of children with COVID-19 or MIS-C requiring surgical consultation for severe gastrointestinal symptoms.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.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.

BACKGROUND: This study reports the feasibility, validity, and reliability of the Turkish versions of the Esophageal-Atresia-Quality-of-Life (EA-QOL) questionnaires, which were originally developed in Sweden and Germany. METHODS: After translation from Swedish to Turkish and cognitive debriefings, 51 families of children aged 2-7 years (parent-reported, 17 items) and 54 families of children 8-17 years (child-reported and parent-reported, 24 items) responded to the EA-QOL questionnaires and a validated generic HRQOL instrument (PedsQL4.0). The medical records of the patients and the questionnaires were used to obtain clinical data. The Turkish version of the EA-QOL questionnaires were evaluated for feasibility (

© 2022 Wiley Periodicals LLC.Objectives: Postoperative ventilatory strategies in patients with esophageal atresia (EA) and tracheoesophageal fistula (TEF) may have an impact on early postoperative complications. Our national Esophageal Atresia Registry was evaluated to define a possible relationship between the type and duration of respiratory support on postoperative complications and outcome. Study Design: Among the data registered by 31 centers between 2015 and 2021, patients with esophago-esophageal anastomosis (EEA)/tracheoesophageal fistula (TEF) were divided into two groups; invasive ventilatory support (IV) and noninvasive ventilatory support and/or oxygen support (NIV-OS). The demographic findings, gestational age, type of atresia, associated anomalies, and genetic malformations were evaluated. We compared the type of repair, gap length, chest tube insertion, follow-up times, tensioned anastomosis, postoperative complications, esophageal dilatations, respiratory problems requiring treatment after the operation, and mortality rates. Results: Among 650 registered patients, 502 patients with EEA/TEF repair included the study. Four hundred and seventy of patients require IV and 32 of them had NIV-OS treatment. The IV group had lower mean birth weights and higher incidence of respiratory problems when compared to NIV-OS group. Also, NIV-OS group had significantly higher incidence of associated anomalies than IV groups. The rates of postoperative complications and mortality were not different between the IV and NIV-OS groups. Conclusion: We demonstrated that patients who required invasive ventilation had a higher incidence of low birth weight and respiratory morbidity. We found no relation between mode of postoperative ventilation and surgical complications. Randomized controlled trials and clinical guidelines are needed to define the best type of ventilation strategy in children with EA/TEF.

Congenital esophageal web is a very rare type of esophageal atresia (EA) and is considered as Type IV atresia in Kluth's classification. Type IV EA is further classified into subgroups according to the location of web and the presence of tracheoesophageal fistula (TEF). Herein, we present a newborn having Type IV1a EA who was initially suspected as isolated EA and has co-existing malrotation together with the review of the literature. A newborn having congenital esophageal web was admitted with antenatal ultrasonography revealing polyhydramnios, dilated stomach, and duodenum. Abdominal X-ray revealed a gasless abdomen suggesting isolated EA. The upper gastrointestinal series confirmed complete obstruction at the lower esophagus. Bronchoscopy performed excluding TEF. Laparotomy revealed intestinal malrotation and the absence of another GI webs. Gastrotomy was performed and mucosal web at GEJ was seen by pushing nasogastric catheter. Web excision and Ladd procedure were performed. Congenital esophageal web is an extremely rare type of esophageal anomaly that can be only classified in Kluth classification. A high index of suspicion is required for diagnosis especially in newborns with the gasless abdomen and esophageal obstruction at the level of GEJ. Preoperative bronchoscopy is mandatory to exclude an associating TEF. Surgical exploration via laparotomy is beneficial for exploring additional GIS anomalies.

Introduction This article assesses (1) access to splenic embolization (SE), (2) indications for SE, and (3) post-embolization management in high-grade splenic trauma in children. Materials and Methods An online questionnaire was sent in 2021 to all members of European Pediatric Surgeons' Association. Results There were a total of 157 responses (50 countries, 83% academic hospitals). Among them, 68% have access to SE (SE) and 32% do not (nSE). For a hemodynamic stable patient with high-grade isolated splenic trauma without contrast extravasation (CE) on computed tomography (CT) scan, 99% SE and 95% nSE respondents use nonoperative management (NOM). In cases with CE, NOM decreases to 50% (p = 0.01) and 51% (p = 0.007) in SE and nSE centers, respectively. SE respondents report a significant reduction of NOM in stable patients with an associated spine injury requiring urgent surgery in prone position, both without and with CE (90 and 28%, respectively). For these respondents, in stable patients the association of a femur fracture only tends to decrease the NOM, both without and with CE (93 and 39%, respectively). There was no significant difference in NOM in group nSE with associated injuries with or without CE. After proximal SE with preserved spleen vascularization on ultrasound Doppler, 44% respondents prescribe antibiotics and/or immunizations. Conclusion Two-thirds of respondents have access to SE. For SE respondents, SE is used even in stable patients when CE showed on initial CT scan and its use increased with the concomitant need for spinal surgery. There is currently a variation in the use of SE and antibiotics/immunizations following SE.

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.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.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 IThis 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.

Gastrointestinal symptoms appear to be one of the most common presentations of SARS-CoV-2 infection, later named as COVID-19. The symptoms such as nausea, emesis, abdominal pain, and diarrhea may be recognized as either a finding of COVID-19 or prominent presentation of multi-inflammatory syndrome in children (MIS-C). COVID-19 may present with acute appendicitis and/or may mimic its clinical findings. Although, co-existence of acute appendicitis and COVID-19 has been well documented, it is not clear whether appendicitis is a complication of COVID-19 or coincidence in children. A 13-year-old boy who developed acute appendicitis during the clinical course of COVID-19 is presented to discuss the possibility of causal relationship between appendicitis and COVID-19 in children.

Urorectal septum malformation sequence (URSM) is a rare congenital anomaly of caudal end of mesoderm. There is usually a single or no opening in the perineum. An infant with dysmorphic facial features, ambiguous genitalia, meningomyelocele, pes equinovarus, left renal agenesis, severe hydronephrosis of the right kidney, and anuria requiring right ureterocutaneostomy during the neonatal period is reported. Initial physical examination revealed fusion of labioscrotal folds forming a single sac without a raphe, and a single gonad on the left side of the sac, less than 1 mm in size. There was no phallus, urethral opening, or any palpable cavernous body. Karyotype analysis from peripheral blood was 46XY. Chromosomal microarray analysis using Agilent 8X60K platform revealed arr [hg19](1-22)x2,(X,Y)x1. DNA sequence analysis for WT1 gene was normal. During inguinal hernia repair, cavernous bodies were detected in the retroperitoneal space under the hernia sac intraoperatively. Penis was reconstructed using two rotational flaps from the midline skin above the pubic bone to cover the cavernous bodies. This report aims to discuss the clinical features as well as surgical options for reconstruction of an abnormally located penis in an infant with partial URSM. Level of evidence: Level V, therapeutic study.

A systematic review of the literature was performed to evaluate the results and outcomes of the thoracoscopic bullae and/bleb excision (TBBE) for the treatment of spontaneous pneumothorax (SP) in children. An electronic search of MEDLINE and PUBMED was performed by using the keywords: thoracoscopy’, ‘spontaneous pneumothorax’, ‘bullae’, ‘bleb’ and ‘children’. The selected articles searched for demographic features, localization of blebs and preoperative computed tomography (CT) findings. The operative time, postoperative outcomes (duration of chest tube and length of hospital stay) and complications (air leak, failure and recurrence) were also recorded. Eighteen studies include 814 cases in which 57% (n = 464) of them associated with bullae/bleb. The mean age of patients was 14.8 years. 82.8% of patients were male. The left side was the most common localization. Bullae/blebs were defined in 31.3% (n = 144) of preoperative CT scans. The mean operative time showed a range of 55–85 min depending on the surgical procedure. Five of the studies reported the duration of postoperative chest tube drainage ranging from 3.3 to 25 days. The mean hospital stay was 4.3–10 days. The complications were bleeding (n = 1), postoperative air leaks (n = 6) and Bernard-Horner Syndrome (n = 1). The recurrence rates were reported in 4–30% of all the TBBE procedures. TBBE is a safe and effective treatment for SP in children. TBBE has several advantages over thoracotomy, including less recurrence and complication rates. The timing of the thoracoscopic excision, the best surgical instruments that would lead to less complications, and optimum treatment of recurrence still need to be addressed.

BACKGROUND: The objective of this study is to evaluate the obstetric outcomes of pregnancies with congenital diaphragmatic hernia (CDH). METHODS: Fifty one pregnancies prenatally diagnosed with CDH at our institution between January 1, 2002 and August 31, 2018 were retrospectively evaluated. The pregnancies were divided into two groups according to neonatal survival. Demographic features, clinical characteristics and prognostic factors were compared between the neonatal survival (n = 16) and non-survival (n = 28) groups. Cut-off values of fetal lung area to head circumference ratio (LHR), observed/expected LHR (o/e LHR) and observed/expected total fetal lung volume (o/e TFLV) for neonatal survival were calculated. RESULTS: Thirty six (70.6%) and fifteen (29.4%) fetuses had left and right sided CDH respectively. Seven patients chose termination of their pregnancies (13.7%). Statistically significant differences were found between survival and non-survival groups in terms of parity, median gestational week at diagnosis, polyhydroamniosis rate, CDH type, stomach position, liver position, median LHR, o/e LHR, o/e TFLV, median 5th minute Apgar score and neonatal operation rate values (p values were 0.03

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

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.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.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.The FCT is an effective method to improve chewing function in children EA-TEF who had CD.Single group treatment study.Level 4.

The antegrade continence enema (ACE) procedures are successful surgical options to achieve bowel cleaning in children with faecal incontinence due to a neuropathy or an anorectal malformation and an intractable constipation. The reversed and orthotopic appendicocecostomy, tubularized ileal conduit and the tubularized cecal flap are frequently applied procedures in the treatment of faecal incontinence (FI). The most common complications are the stoma stenosis, leakage, prolapse, adhesive obstruction and the difficulty in catheterization. Each procedure has its own advantages and disadvantages with different complication rates. The postoperative complications of ACE procedures may reduce patient compliance and quality of life. Most of these complications can be preventable and are easy to manage. This review aimed to discuss the prevention and management strategies for various ACE procedure complications.

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.

Aim To evaluate the practice patterns of the European Pediatric Surgeons' Association (EUPSA) members regarding the management of primary spontaneous pneumothorax (PSP) in children. Methods An online survey was distributed to all members of EUPSA. Results In total, 131 members from 44 countries participated in the survey. Interventional approach (78%) is the most common choice of treatment in the first episode, and most commonly, chest tube insertion (71%) is performed. In the case of a respiratory stable patient, 60% of the responders insert chest tubes if the pneumothorax is more than 2 cm. While 49% of surgeons prefer surgical intervention in the second episode, 42% still prefer chest tube insertion. Main indications for surgical treatment were the presence of bullae more than 2 cm (77%), and recurrent pneumothorax (76%). Eighty-four percent of surgeons prefer thoracoscopy and perform excision of bullae with safe margins (91%). To prevent recurrences, 54% of surgeons perform surgical pleurodesis with pleural abrasion (55%) and partial pleurectomy (22%). The responders who perform thoracoscopy use more surgical pleurodesis and prefer shorter chest tube duration than the surgeons performing open surgery (p Conclusion Most of the responders prefer chest tube insertion in the management of first episode of PSP and perform surgical treatment in the second episode in case of underlying bullae more than 2 cm and recurrent pneumothorax. The surgeons performing thoracoscopy use more surgical pleurodesis and prefer shorter chest tube duration than the responders performing open surgery. The development of evidence-based guidelines may help standardize care and improve outcomes in children with PSP.

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.

Pyloric atresia (PA) is a rare gastrointestinal anomaly that occurs either as an isolated lesion or in association with other congenital or hereditary anomalies. Familial occurrence of PA with epidermolysis bullosa (EB) has been well documented and variants in ITGA6, ITGB4, and PLEC are known to cause EB with PA. However, no gene variants have been defined in familial isolated PA. Five siblings with familial isolated PA are presented that suggest biallelic ITGB4 variants may underlie the development of PA without EB. Five siblings from two unrelated families with isolated PA were studied with exome sequencing (ES) to identify the genetic etiology in isolated familial cases. Exome sequencing was performed in one affected patient from each family. Validation and segregation studies were done by Sanger sequencing. Parents were first cousins in one family but there was no consanguinity in the other family. Type-2 PA was detected in both families and none of the probands had associated anomalies. All patients underwent successful gastroduodenostomy and have been under follow-up uneventfully. All patients had biallelic ITGB4 variants, c.2032G > T p.(Asp678Tyr) being a novel one. Biallelic ITGB4 variants may underlie the development of PA without associated EB. Further detection of variants in this gene may establish any possible genotype-phenotype correlations.

Congenital esophageal web is a very rare type of esophageal atresia (EA) and is considered as Type IV atresia in Kluth's classification. Type IV EA is further classified into subgroups according to the location of web and the presence of tracheoesophageal fistula (TEF). Herein, we present a newborn having Type IV

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 (

Objective: To evaluate and compare the outcomes of pregnancies with prenatally detected gastroschisis and omphalocele. Materials and Methods: We retrospectively evaluated prenatally detected gastroschisis and omphalocele cases. Cases were compared in terms of maternal demographic and clinical characteristics as well as pregnancy and neonatal outcomes. Results: This study consisted of 17 gastroschisis and 30 omphalocele cases. Only one case with gastroschisis was terminated due to additional severe limb deformities. Seventeen out of 30 cases of omphalocele were terminated for various reasons (56.7%). All patients with gastroschisis had surgical repair, while 8 out of 13 omphalocele cases had surgery. One patient with an omphalocele died after surgery due to sepsis. Six cases of gastroschisis also died in the neonatal period due to various reasons (6/16, 37.5%). Conclusion: Additional genetic disorders are more frequent in those with omphalocele cases, and they are more frequently terminated during gestation that the gastroschisis fetuses.

Background Pulmonary Langerhans's cell histiocytosis (PLCH) is a rare cause of interstitial lung disease in children and more than half of the cases are bilateral. Persistent respiratory distress due to spontaneous pneumothorax (SP) in bilateral PLCH may refractory to conservative treatment and posed a great challenge to surgical modalities. A 3-year-old boy with SP due to bilateral PLCH is presented to discuss the surgical options of recurrent and refractory PLCH cases in children. Case Report The patient was admitted to the emergency department with severe respiratory distress and SP. After chest tube insertion, biopsy from neck mass revealed Langerhans's cell histiocytosis. Chemotherapy including vinblastine and prednisone was initiated. Due to persistent respiratory difficulty and air leaks, talc pleurodesis and thoracoscopic bullae excision with pleural decortication were performed. Two months after the admission, due to nosocomial infection and severe respiratory distress, extracorporeal membranous oxygenation (ECMO) support was initiated. The patient was died of ECMO complications on 24th day of ECMO. Conclusion Despite the use of chemotherapy and surgical excision of cystic lesions, bilateral PLCH in children may have lethal outcome. Other treatment options including respiratory support with ECMO and lung transplantation should be considered as last resort of treatment alternative in persistent cases.

Introduction Recurrent pulmonary infections, wheezing and stridor due to swallowing dysfunction, esophageal dysmotility, gastroesophageal reflux, tracheomalacia and bronchomalacia are frequently seen complications after esophageal atresia and tracheo-esophageal fistula (EA-TEF) surgeries. This study aimed to investigate the frequency and causes of respiratory problems and to evaluate the factors that affect respiratory morbidity in patients who had undergone EA-TEF repair in a tertiary referral center. Methods Preoperative and postoperative records of patients with EA, TEF + EA and isolated EA were examined retrospectively. Accompanied diseases and swallowing dysfunction symptoms were questioned. Bronchoalveolar lavage results were investigated if the patient had flexible bronchoscopy. Results A total of 71 children with EA were included in the study, and seven patients who did not have follow-up after surgery were excluded. 46 of the 64 patients continue regular follow-up visits in our department. Male sex, primary EA repair in another center, EA type C, accompanying genetic anomalies, severe tracheomalacia, late per oral feeding (1 year after surgery), and severe GER were found to cause significantly higher incidence of coughing, recurrent wheezing, recurrent pneumonia, and bronchiectasis despite surgical and medical treatments (p = 0.048, p = 0.045, p = 0.009, p = 0.029, p = 0.025). Conclusion Even if anatomical anomalies are corrected by surgery in patients who underwent EA repair, precautions can be taken for GERD, laryngotracheomalacia, and swallowing dysfunction, and effective pulmonary rehabilitation can be initiated with early multidisciplinary approach before the development of respiratory tract symptoms.

Esophageal replacement (ER) procedures have been widely used to replace the native esophagus over the past few decades. Although satisfactory results have been reported in different ER procedures, controversies exist about the best surgical technique with least complications. There is limited number of comparative studies evaluating the results of different ER techniques. Most of these studies include data from heterogeneous group of patients with different indications. Therefore, without randomized controlled studies, it is difficult to make a strong recommendation about the best surgical option. The previous studies demonstrate that minor complications are more common after colonic substitutions (CS) than gastric pull-up (GPU) procedures, whereas major complications are more frequent after GPU’s. In a meta-analysis, CS was shown to be associated with gastrointestinal complications, but fewer respiratory complications than GPU. The results of JI are highly divergent and commonly depend on the experience of surgeons and centers. Finally, mortality rates associated with different surgical techniques were similar.

INTRODUCTION: Objective: A retrospective study was planned to evaluate the clinical and radiological findings of childhood branchial anomalies together with the subclasses of the anomaly and to discuss the surgical results. Method: The medical records of the patients managed for branchial cleft anomaly between 2014 and 2019 were examined. Demographic features, clinical and radiological findings, surgical managements and outcomes were recorded. Results: Eighteen cases were included in the study. Median age of the cases was 5 years (1-14). Male/female ratio was 9/9. The complaints were discharge from the BC (n=13, 72%) and painless neck mass (n=3, 17%). The BC was at right side in 8 (44%), left side in 9 (%50) and bilateral in 1 case (6%). Physical examination revealed the presence of a fistula, and its orifice in 14 cases and cystic lesion was palpable in 4 cases. Ultrasound was performed in 9 cases (50%) and cyst could be detected in 3 of them. All cases were operated and step-ladder incision was used in 8 cases. The fistula tract was ended near to pharynx in 2, near to pretonsillar fossa in 10, submandibular region in 5, and external auditory tract in 1 case. Histopathological examination revealed inflammation in 2 cases and cartilage in 1 case. Stratified ciliary columnar and stratified squamous cell epithelium was detected in samples. Conclusion: Physical examination is the most important diagnostic method. The management is surgery and excision of whole lesion is important in preventing recurrences. Although using methylene blue is a guiding technique in surgical excision of tracts, the effect of its use on surgical outcome and recurrence rates is still debatable.

Introduction The data of the Turkish Esophageal Atresia Registry (TEAR) was evaluated to define the outcome of very low birth weight (VLBW) and low BW (LWB) infants with esophageal atresia (EA). Materials and Methods The data registered by 24 centers between 2014 and 2018 were evaluated for demographic features, prenatal findings, associated anomalies, surgical treatment, and outcome. Patients were enrolled in three groups according to their BWs (VLBW 2,500 g). Results Among the 389 cases, there were 37 patients (9.5%) in the VLBW group, 165 patients (42.4%) in the LBW group, and 187 patients (48.1%) in the NBW group. Prenatal diagnosis rates were similar among the three groups (29.7, 34.5, and 24.6%, respectively). The standard primary anastomosis was achieved at a significantly higher rate in NWB cases than in the other groups (p Conclusion The national data of TEAR demonstrates that the developmental and feeding parameters are better in NBW patients. Although VLBW patients have higher risk of developing fistula canalization than the LBW and NBW groups, long-term complications, such as anastomotic strictures, weight, and height values, after 1 year are similar in both groups. According to our results, associated anomalies and LBWs are still significant risk factors for mortality in cases with EA.