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A tumor with EWSR1/FLI fusion displaying extensive well differentiated neuroblastomatous differentiation is presented.A nine-year-old female patient had a thoracic vertebra 8 paraspinal mass. The lesion was resected incompletely. Histopathologically, a small round cell tumor with gangliomatous differentiation was seen. This was initially diagnosed as an intermixed ganglioneuroblastoma. In the completion surgery biopsy material, the small round cell component was more prominent. Immunohistochemistry for both samples showed membrane positivity for CD99 and nuclear positivity for NKX2.2 in the small round cell component of the tumor. Molecular analysis revealed EWSR1/FLI fusion. The diagnosis then considered a "Ewing Sarcoma Displaying Extensive Well Differentiated Neuroblastomatous Differentiation".Tumors with the EWSR1/FLI fusion may show neuroblastomatous differentiation. We chose to treat this as an Ewing Sarcoma (ES). Recognition of this phenomenon in ES cases may prevent a possible misinterpretation and a failure in oncologic treatment.

Purpose: We examined the relationship between inflammatory reactions in the intestine in Hirschsprung's disease (HD) and Hirschsprung-associated enterocolitis (HAEC). Methods: There were 30 cases (M:27, F:3) who were operated with the transanal endorectal pull-through (TERP) method for HD between 2012 and 2022. The cases were divided into three groups. Group 1: Cases with preoperative and postoperative enterocolitis, Group 2: Cases with postoperative enterocolitis, Group 3: Cases with preoperative enterocolitis. The intestinal segments removed during the surgery were evaluated for interleukin 1 beta (IL-1ß), tumour necrosis factor alpha (TNF-α), and interleukin 6 (IL-6). Results: In the compression of enterocolitis groups and control group, the average intensity of IL-ß staining percentages in lamina propria(LP) were 52,2±23,8% to 17,8±25,4% and in epithelium 50±23,6% to 22,1±24,6%. Those for TNF-α score of LP staining were 65,4±24% to 47,1±24,2%. It has been found that an increase of IL-1ß intensity in LP(p:0.002) and epithelium(p:0.014) of the positive ganglion side is related to enterocolitis. However, a similar relationship for TNF-α was found in only transitional zone LP(p:0.01). Conclusion: It has been shown that endogenous TNF-α secretion and IL-1β-related inflammation play a role in the etiology of HAEC. It has been thought that HAEC is not just an infective disease but an autoinflammatory process.

© 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.

Tubular esophageal duplication is a rare congenital malformation The surgical treatment of this lesion can be challenging. We aimed to present our experience in two cases with tubular esophageal duplication. Both cases were endoscopically treated by a dual knife. The first patient underwent a single, while the second patient required three sessions of endoscopic septum division using an electrosurgical knife. Both showed satisfactory clinical, radiological and endoscopic response to treatment. Moreover, a systematic literature review has been performed. To identify all available studies, a detailed search on tubular esophageal duplication was performed according to the PRISMA (Preferred Reporting Items for Systematic reviews and Meta-Analyses) guidelines. Tubular esophageal duplication was reported in 16 pediatric cases in 14 studies in the literature. Surgery was applied to 10 of these cases and endoscopic treatment was applied to two of them. In the follow-up, nine patients who underwent surgical treatment and two patients who underwent endoscopic treatment were uneventful. Tubular esophageal duplications can be successfully treated endoscopically. Endoscopic septum division is a minimally invasive procedure that allows full anatomical recovery and satisfactory therapeutic response.

Background/aim The criteria for surgical management of ureteropelvic junction obstruction are not well-defined, and there is a risk for loss of renal function before the operation. In this context, certain changes in contralateral kidney had been investigated in order to increase the sensitivity of diagnosis. In this study, we aimed to investigate whether contralateral transient minimal hydronephrosis (CTMH) can be considered as an “early alarm” sign for worsening of the affected kidney in infants with hydronephrosis. Materials and methods A total of 182 infants (92 surgically treated and 90 conservatively followed-up) with unilateral hydronephrosis were retrospectively analyzed. Ultrasonography and renal scan findings were evaluated. Correlation between the appearance of CTMH, contralateral compensatory hypertrophy (CCH) on ultrasonography, and prognosis of the affected kidney were evaluated. Results Among the surgically treated patients, 18 (19.6%) patients developed CTMH on average 7 months (0–13 months) before surgery. Among these 18 patients with CTMH, 12 patients (66.6%) had loss of renal function preoperatively, while this ratio was 29.7% on their counterparts (p = 0049). CCH was observed in 31 (33.7%) individuals in surgically treated patient group including all 18 patients with CTMH, while none of the conservatively followed-up patients developed CCH and/or CTMH. In the multiple logistic regression analysis, among the variables investigated, CTMH was found as an independent predictor of the deterioration in the affected kidney and of the poor prognosis (p = 0.011 and p = 0.0004, respectively). Conclusion In our study, among the variables investigated, CTMH was found as an independent predictor of the deterioration in the affected kidney and poor prognosis in infants followed-up with isolated unilateral hydronephrosis. Additionally, CTMH can be considered as an “early alarm” sign for worsening of the affected kidney and the need for surgical intervention.

Amac: Kongenital diafragma hernili KDH “cok yuksek risk olgularin tani ve tedavilerinin geriye donuk degerlendirilmesidir. Hastalar ve Yontem: Klinigimizde 2005-2015 yillari arasinda takip ve tedavileri yapilan KDH tanili olgularin kayitlari geriye donuk incelendi. Klinigimizde 2005 oncesi modifiye ventilasyon indeksi MVI 80 ve uzerinde olan olgular yuksek riskli olgular olarak tanimlanmaktaydi. Sonraki donemde prenatal tani haftasi

Objectives: To evaluate the surgical approach to pediatric breast masses.Patients and Methods: Patients who underwent a surgical intervention due to breast mass between the years of 2005-2017 were evaluated retrospectively. They were evaluated in terms of age, primary diagnosis, radiological characteristics, surgical treatment method, complications, length of hospital stay and follow-up.Results: The mean age of the 29 cases that were operated on for a breast mass was 14.86 years 6-17 years . The most common complaint at admission was a palpable breast mass. Two cases had bloody discharge from the nipple. The mean duration of the symptoms was 32.3 weeks 2 weeks-1 year . Six cases 20.6% had a family history associated with breast disease. The mean BMI was 18.8 . In 15 patients, the mass was in the left breast, whereas in 13 patients, the mass was in the right breast. However, in one case, two masses were localized bilaterally.Regarding radiological examination, ultrasonography USG was performed on all patients while magnetic resonance imaging MRI was obtained as a supplement in five cases. The mean size of the masses measured radiologically was 2.9 cm 0.9-7 cm . There were multiple lesions in five cases. Needle biopsy was performed in six cases with the suspicion of malignancy in the preoperative period, and they were all found to be consistent with fibroadenoma.Histopathological examination was performed after the surgical excision and revealed fibroadenoma n:26 , borderline phylloides n: 1 , nipple adenoma n: 1 and galactocele n: 1 .The mean follow-up period was 36.6 months 3 months-10 years . New lesions were developed ipsilaterally in three cases and contralaterally in one case during follow-up. Conclusion: Most of pediatric breast masses are benign. Although malignancy is rarely encountered, MRI and needle biopsy are required for accurate surgical planning in case of suspicious ultrasonography findings

Altinay-Kirli E, Ozcan R, Oncul M, Ozmen E, Elicevik M, Buyukunal C, Emir H, Topuzlu-Tekant G. A rare cause of abdominal pain: Ectopic ovary and intestinal malrotation. Turk J Pediatr 2017; 59: 699-703. Ectopic ovary is a rare anomaly that can be associated with unicornuate uterus and renal anomalies. Intestinal rotational anomalies are failure of normal rotation and this arrest in development can predispose to develop a malfixated midgut that is a risk factor for volvulus and significant morbidity and mortality especially in early childhood. Cyclic abdominal pain is a common symptom for both of two distinct pathologies in adolescent ages. Here, we report a case of unicornuate uterus together with right ectopic ovary and intestinal malrotation.

Testotoxicosis is a rare disorder which presents as isosexual peripheral precocious puberty in males. Despite the pattern of autosomal dominant inheritance, sporadic cases also may occur. Due to activating mutation in luteinizing hormone (LH))/choriogonadotropin receptor (LHCGR) gene, early virilization and advancement in bone age are common with increased serum testosterone levels above adult ranges, despite low LH and follicular-stimulating hormone (FSH) levels. There are different treatment regimens, such as combination of bicalutamide (antiandrogen agent) and a third-generation aromatase inhibitor, that are reported to be well-tolerated and successful in slowing bone age advancement and preventing progression of virilization. We report here two patients who presented with peripheral precocious puberty and an activating mutation in the LHCGR gene: one with a family history and previously determined mutation and the other without family history and with a novel mutation (c.830G>T). Combination of bicalutamide+anastrozole was ineffective in slowing pubertal progression and bone age. Short-term results were better with ketoconazole.

Amaç: Koroziv maddeye bağlı özofagusyanığı oluşan olgularda özofagus darlığı gelişimi açısından farkolabilmektedir. Bu çalışmada, özofagus darlığı oluşumunda Human LökositAntijen (HLA) tipine göre bir farklılık olup olmadığının araştırılmasıamaçlandı. Gereç ve Yöntem: Koroziv madde alımı sonrası yapılanilk endoskopide grade 1 ve üzeri özofagus yanığı saptanan olgular geriye dönükolarak değerlendirildi. Grup 1 özofagus darlığı gelişen 10, Grup 2 ise özofagusdarlığı gelişmeyen 10olgudan oluşturuldu. Tüm olgulardan kan örneği alınarak HLA antijentiplendirmesi ve istatistiksel analiz yapıldı.Bulgular: Yaş ortalaması 4,75 yıl (2-10 yıl) idi.HLA antijenlerine göre; grup 1’de HLA-DRB1’11 antijeni, grup 2’de ise HLA-A3antijeni sayısal olarak daha yüksek bulundu. Ancak istatistiksel olarak anlamlı değildi.Sonuç: Çalışmada iki grup arasında HLA antijenleri açısındananlamlı fark bulunamamıştır. Ön rapor niteliğindeki bu çalışma, HLA özofagus darlığı arasındaki ileriçalışmalara yol gösterici olabilir. Olgu sayısının artırılması ile koroziv özofagus darlıklarının erken tanı ve tedavisindeHLA antijenlerinin rolü hakkında daha kesin bilgiler edinilebileceğinidüşünmekteyiz., Objective:Development of esophageal stricture due to corrosive ingestion may be relatedto different factors. In this study it has been aimed to detect if there is adifference esophageal stricture according to Human Leucocyte Antigen (HLA)type.Material and Method: The patients who were diagnosed with grade 1 orhigher esophageal burn has been evaluated retrospectively. Group 1, consistedof 10 cases who have developed esophageal stricture and group 2, consisted of10 cases who have not. Blood samples were taken from all of the patients forHLA antigen typing and evaluated with statistical analysis.Results: Meanage was 4.75 (2-10) years. HLA-DRB1’11 was detected more common in group 1 andHLA-A3 in group 2. This difference was neither significant statistically forboth.Conclusion: We did not find any significant difference in HLA antigentypes between Group 1 and Group 2 about stricture development; however, thisearly report can predict a guide for further studies. With an increase in casenumber, we think that more precise information can be acquired about the roleof HLA in diagnosis and treatment of esophageal strictures.

Summary: Objective: To evaluate the outcome and characteristics of patients who were operated for sacrococcygeal pilonidal sinus (SPS) treatment using primary repair or secondary healing technique. Methods: Forty-seven patients (female: 25, male: 22) diagnosed with pilonidal sinus operated between 2009 and 2015 were retrospectively analyzed. The cases were evaluated for age, gender, body mass index (BMI), surgical technique, hospital stay, recovery time and recurrence. Mainly two types of surgical techniques were applied: primary repair and secondary healing. Results: Mean age of the patients was 15.6 (±1.2) years. Excision and primary repair was performed in 36, excision and secondary healing was in 11. There was no statistically significant difference between the groups regarding length of hospital stay and duration of postoperative pain (p > 0.05). There was a statistically significant difference between groups regarding recovery time (p

Background The effectiveness of the TAP block in children has been well characterized in literature. However, there are only few reports about TAP block in the neonates and low birth weight groups. This is a retrospective observational analysis of ultrasound - assisted TAP blocks in neonates and young infants. The aim of this study to analyze retrospectively the analgesic effectiveness of TAP block in neonates and infants undergoing abdominal and inguinal surgeries. Methods Thirty-four cases of neonates and infants with (whom) applied TAP block were retrospectively analyzed. The TAP block was performed postoperatively in supraumbilical surgeries and preoperatively in infraumbilical surgeries. The TAP block was applied with 0.8 ml/kg-1 of 0.25% bupivacaine in unilateral approach and 1.6 mlkg-1 of 0.125% bupivacaine in bilateral approach. The CRIES Pain Scale was used for postoperative pain measurement of neonates. Results The patient's age ranged from 2 to 88 day-old with a mean (SD) of 36.2(24.2). Eleven of them were premature babies. The weight ranged from 1.6 to 5.8 with a mean (SD) of 3.7kg (1.1). Twenty-nine patients were extubated at the end of the surgery and the other patients within 12 hours. 67.7% infants required no additional postoperative analgesic in 24 hours and none of them required narcotic analgesics. Conclusions Our conclusion is that the use of TAP blocks results in low analgesic requirements and a low incidence of postoperative intubation and mechanical ventilation in neonates and infants. It should be considered in this age group of child for postoperative analgesia.

Amaç: Çocukluk çağı solidtümörlerinin akciğer metastazlarında kliniğimizin cerrahi yaklaşımınındeğerlendirilmesidir.Olgular ve Yöntem: Akciğer metastazı nedeniyle 1978-2016 arasında başvuran ve cerrahigirişim yapılan olgular geriye dönük olarak incelendi. Yaş, cinsiyet, primertanı, akciğer metastazının ortaya çıkış zamanı, cerrahi tedavi yöntemi,patolojik tanı ve takip açısından değerlendirme yapıldı. Primer tümörü kontrolaltına alınan, başka uzak organ metastazı olmayan ve akciğer metastazı içincerrahi tedavi uygulanan olgular çalışmaya alındı.Bulgular: Onsekiz olgunun (K/E:11/7)yaş ortalaması 8,1 yıl (1,5-14 yaş) idi. Primer tümörler; Wilms tümörü(n:9,%50), Ewing sarkomu (n:3,%17), osteosarkom (n:2,%11), hepatoblastom(n:1,%5,5), fibrosarkom (n:1,%5,5), rabdomyosarkom (n:1,%5,5) ve endodermalsinüs tümörü (n:1,%5,5) idi. Olguların 4’ünde ilk başvuru anında akciğermetastazı mevcuttu. Onaltısında metastazın ortaya çıkış zamanı ortalama 16, 5ay (7ay-4 yıl) idi. Radyolojik değerlendirme tüm olgularda preoperatif dönemdeakciğer grafisi ve bilgisayarlı tomografi (BT) ile yapıldı. Onsekiz olguyatoplam 23 torakotomi yapıldı. Yirmibir wedge rezeksiyon, 1 lobektomi, 1lobektomi ve kot rezeksiyonu uygulandı. Patolojik incelemede çıkarılanlezyonların 3’ü inflamatuar reaksiyon olarak değerlendirildi. Toraks drenialınma süresi ortalama 5,2 gün(3-8), hastanede kalış süresi 7,3 gün(5-10 gün)idi. Cerrahi girişime bağlı komplikasyon görülmedi. Takipte 6 olgu ilerleyiciprimer hastalık nedeniyle kaybedildi.Sonuç: Çocukluk çağı tümörlerindeakciğer metastazları başvuru sırasında ve/veya takipte görülebilir. Cerrahitedavisinde sınırlı rezeksiyon yeterli görünmektedir. Torakotomi bu olgulardatüm lezyonların değerlendirilmesini sağlamaktadır ve güvenle uygulanmaktadır.Radyolojik incelemeler tümör metastazını desteklese de kesin tanı patolojikinceleme ile konulmaktadır., Aim: To evaluate our surgicalapproach to lung metastasis of childhood tumors.Patients and method: Cases with solid tumorlung metastasis that underwent surgical intervention between years 1978-2016were investigated retrospectively. Patients were evaluated regarding age,gender, primary diagnosis, time of lung metastasis occurence, surgicaltreatment method, pathological diagnosis and follow-up.Results: Of the 18 cases which wereoperated due to lung metastasis 11 were female, 7 were male. Median age was 8,1years (range 1,5-14 years). The most common pulmonarymetastasis were related to Wilms' tumor(n:9). Lung metastasis were present at theinitial diagnosis in 4 patients. In the remaining 14 patients, the average timeof occurence of metastasis was 16,5 months ( range 7 mo-4yr). In all cases theCT imaging demonstrated findings compatible with lung metastasis. Twenty-three thoracotomies were performed in eighteen cases. Surgical procedures were;wedge resection in 21, lobectomy in 1 and lobectomy with costal bone resectionin another. Pathological diagnosis was negative for tumor metastasis in 3patients who had CT imaging indicative of the disease. Average time of chesttube removal was 5,2 days (range 3-8 ) while average length of hospital staywas 7,3 days (range 5-10 days). During follow-up, 6 cases died due toprogressive primary disease.Conclusion: In patients with childhoodtumors, lung metastasis can be encountered at initial diagnosis and/or duringfollow-up. From the surgical point of view, limited resection is considered tobe adequate. Although radiological imaging supports tumor metastasis,definitive diagnosis should be made via pathological evaluation.

Summary Objective The aim was to analyze testis-sparing surgical procedures in boys with Leydig cell pathologies. Study design The hospital records of four boys with Leydig cell hyperplasia who underwent testis-sparing surgery for testicular masses between 2000 and 2012 were analyzed retrospectively. Tumor markers were evaluated and all boys underwent scrotal ultrasonography preoperatively. The hormonal profile was also analyzed for symptoms of precocious puberty. The testis was delivered through a high transverse inguinal incision and the tumor was excised by enucleation. After confirming the benign nature of the tumor with frozen-section examination, the testis was reinserted and fixed into the scrotum with absorbable sutures. All cases were followed-up with physical examination, scrotal ultrasonography, and measurement of β-human chorionic gonadotropin (HCG), α-fetoprotein, and hormone levels. Results The mean age of the patients was 9.4 years (1.5–15 years). Testicular mass and scrotal asymmetry were detected in all cases. Ultrasonography was the main initial diagnostic modality for detecting testicular masses ( Table ). β-HCG and α-fetoprotein levels were normal. Three cases had Leydig cell hyperplasia and one patient was diagnosed to have a Leydig cell tumor. Signs of precocious puberty were detected in the four patients. The mean follow-up period was 4.8 years (2–8 years). Neither recurrence nor testicular atrophy developed in the follow-up. Findings of precocious puberty continued in one patient with Leydig cell hyperplasia, in whom a 2-mm contralateral metachronous lesion was detected and enucleated successfully. Discussion Testis-sparing surgery with its potential long-term psychological, cosmetic, and functional advantages should be used in pediatric patients in whom a benign Leydig cell pathology is confirmed histopathologically. Conclusion This intervention with good long-term results can easily be applied through a proper dissection plane in the testicle. Since testicular Leydig cell tumors in childhood have small rates of recurrence, this choice of treatment is efficient in patients with salvageable testicular tissues and normal levels of tumor markers. Table . Laboratory and ultrasound findings with final diagnosis. Case Age (years) Side Preoperative levels Testis volume (mL) Diagnosis Follow-up period (years) AFP β-HCG Testosterone Contralateral testis volume Testis volume with mass 1 10 Right N N Total: 531 ng/dL Free: N/A 1.4 2 Leydig cell hyperplasia 3 2 15 Left N N Total: 440 ng/dL Free: 3.91 pg/mL 12 14 Leydig cell hyperplasia 9 3 a 1.5 Metachronous N N Total: 842 ng/dL Free: 2.6 pg/mL 1.2 1.6 Leydig cell hyperplasia 3 4 11 Left N N Total: 125.4 ng/dL Free: 0.6 pg/mL 1.4 1.8 Leydig cell tumor 4 Note . Normal (N) ranges: total testosterone (2–32 ng/dL); free testosterone (3.84–34.17 pg/mL). AFP = α-fetoprotein; β-HCG = β-human chorionic gonadotropin. a Enucleation was performed in case 3 because of a subsequent contralateral tumor.

To share our experience in ovary-sparing surgery for teratomas in children. The medical records of nine patients (mean age of 11.2 years, r 6–15 years) who had undergone ovary-sparing surgery for teratoma were analyzed retrospectively. Mean duration for follow-up was 29.5 months (r 15–75 months). Five patients suffered from chronic abdominal pain; two had acute colicky abdominal pain. In two patients, there was no presenting clinical symptom. Two patients were operated on emergency basis due to symptoms related with acute abdomen. On the other hand, seven were operated electively. Ultrasonography was performed in all patients. Additionally, MRI and tumor markers were performed in all but two. Main radiologic findings consisted of heterogenous cystic and solid ovarian masses predictive of teratoma. The definitive diagnosis in emergency cases were as follows: perforated appendicitis plus teratoma [1]; torsion of the ovarian mass with teratoma [1]. The final diagnosis in electively treated seven patients were: unilateral ovarian teratoma [4], bilateral ovarian teratoma [1], bilateral teratoma plus appendiceal inflammatory mass [1], unilateral teratoma and contralateral corpus hemorrhagicum cyst [1]. The operations were performed by open conventional surgery in six and laparoscopy in three patients. The procedures were ovary-sparing surgery in 12 ovaries, appendectomy in 2 patients and detorsion of ovary in 1 patient. The main indication for ovary-sparing surgery was the “existence of a perfect dissection plane between the tumor margins and healthy ovarian tissue”. The remaining ovarian tissue was evaluated macroscopically for residual lesions. Frozen section was performed in three suspected patients and the ovarian margins were free of any tumor cell. The pathologic diagnosis was: mature cystic teratoma in 10, immature teratoma in 1 and corpus hemorrhagicum cyst in 1. The postoperative outcome and follow-up was uneventful. Heterogenous ovary mass containing solid and cystic portions with echogenic areas on ultrasound imaging is highly suggestive of ovarian teratomas. Emergent surgical intervention is indicated if there is any suspicion of ovarian torsion. Otherwise, MRI is performed for further radiological evaluation. Based on radiologic findings, ovary-sparing surgery can be safely performed if the preoperative diagnosis is teratoma and there is always a plane of dissection between the normal ovary and cyst wall.

Amac: Sunnet cocuklarda en sik uygulanan cerrahi girisimlerden biridir ancak kanama bozuklugu olan cocuklarda sunnetle ilgili az sayida calisma vardir Bu calismada kanama sorunu olan cocuklarda sunnetle ilgili deneyimlerimizi paylasmayi amacladik Gerec ve Yontem: Cerrahpasa Tip Fakultesi Cocuk Hematoloji Bolumu rsquo;muzde kanama bozuklugu nedeni ile izlenen ve Cerrahpasa Tip Fakultesi Cocuk Cerrahisi Bolumu rsquo; rsquo;nde 2000 2010 yillari arasinda sunnet yapilan 18 olgunun yaslari 3 14 dosyalari geriye donuk olarak incelendi Tedavi yontemleri komplikasyonlar ve sonuclar degerlendirildi Bulgular: Olgularin yedisinde hemostazin birinci evresini ilgilendiren sorun vardi: Ucu Glanzmann trombastenisi ikisi Bernard Soulier sendromu ikisi kronik immun trombositopenik purpura idi Diger 11 olgu ise hemostazin ikinci evresini ilgilendiren nedenlerle izlemde olan olgulardi: Uc von Willebrand hastaligi vWH bir faktor V eksikligi bir hemofili B ve ikisi inhibitorlu olan alti hemofili A hastasi Trombosit sayi ve veya islev bozuklugu olan hastalarin hicbirine trombosit verilmedi Sadece antifibrinolitik ve yerel fibrin yapistiricilarla islem sorunsuz olarak gerceklestirildi ve kanama olmadi Hemofili inhibitorsuz ve vWH olan olgularda ise sunnet oncesinden baslatilan 2 3 faktor 15 25 kg gun tedavisine sunnetten sonra 24 48 saat devam edildi Antifibrinolitik tedavisi ve fibrin yapistirici da kullanilarak hicbir hastada kanama gorulmedi Iki dusuk duzeyde inhibitorlu olguda yogun ldquo;bypas rdquo; ajanlari kullanildi 12 ve 18 gun sure ile Kanama ancak rFVIIa ve aPCC rsquo;nin ardisik kullanimi ile durdurulabildi Cikarimlar: Kanama bozuklugu olan cocuklar deneyimli bir merkezde guvenle sunnet edilebilirler Kanama bozuklugu olan cocuklarin sunnetinde antifibrinolitik ve yerel hemostatik maddelerin kullanimi ile hemofilik hastalarin faktor dozlari azaltilabilir ve trombosit bozuklugu olan hastalarda trombosit transfuzyonundan kacinilabilinir Turk Ped Arfl 2011; 46: 313 7

Aim: To evaluate the outcomes of management of ureteropelvic junction obstruction in premature patients by comparing them to a group of non-premature. Material and Methods: We reviewed the medical records of 102 patients with isolated hydronephrosis which were referred from pediatric nephrology outpatient clinic or those who admitted directly to pediatric urology clinic under one year of age between the years of 2005 and 2016. Two groups were set up; premature and term. The gestational age, sex, management for ureteropelvic junction obstruction, complications were recorded. Chi-Square, Fisher’s and Mann-Whitney-U tests were used for statistical analyzes. Results: There were 92 patients in the term group and 10 in the premature group. All patients were operated. Preoperative symptoms such as pyonephrosis, renal calculi, and hypertension were relatively common in the premature group. Preoperative low function, postoperative loss of function, and poor outcome were statistically more common in premature patients. Conclusion: In our limited experience, prematurity is an independent factor associated with UPJO, and it may indicate a poor prognosis. We advocate that this issue should be considered in practice.

Akut kar›n a¤r›s› ile daha onceleri bilinmeyen ve bir haftadan daha yeni olan kar›n a¤r›s› tan›mlanmaktad›r (2). Baflka sistemik ve/veya gastrointestinal semptomlar ve lokal bulgular da hastal›k tablosuna eklenirler. Bu bulgular›n klini¤e yans›mas›; 1. A¤r› 2. ‹fltahs›zl›k, bulant› ve kusma, 3. Distansiyon, kab›zl›k veya ishal fleklindedir. 1. A¤r› Kar›n a¤r›s› uc grupta incelenir: Visseral (splanknik) a¤r› • Kar›n icindeki ici bofl organlar›n spastik kas›lmalar›, intraluminal bas›nc art›fl›, iskemi ve solid organlar›n kapsullerinin ani gerilmesi sonucu oluflur. • A¤r›n›n bafllang›c› yavafl ve suresi uzundur. • Hasta a¤r›y› iyi tarif edemez, kunt bir a¤r›d›r. • A¤r›n›n lokalizasyonu zordur. • Otonom C grubu sinirler a¤r›y› iletir. Bu afferent sinir lifleri ile merkezi sinir sistemine iletilen a¤r› ayn› dermatoma uyan alanda hissedilir. Bu nedenle visseral a¤r›; on barsak Ca¤r›l› Yazar Invited Author

To analyse patients those admitted to our clinic due to perforation in duodenum. Fourteen patients those have been admitted to our center with perforation in duodenum between 1990 - 2014 analysed retrospectively. Ten patients (8M, 6F) those have been admitted to our clinic between 1990 - 2014 have a mean age of 6.2 years (25 days - 16 years). Two of cases admitted directly to our clinic and the rest referred from another hospitals. Mean time for appliance to our clinic was 3.2 days (1day - 1 week). Ulcer in duodenum was the cause of perforation in 10 cases while in 3 the cause was trauma and in 1 case was surgical complication of infantly persistant hyperinsulinism (IPHH). The case with hyperbilluribinemia after near total pancreatectomy due to IPHH had been explored and perforation in deuedenum diagnosed. Resection in first two parts of duodenum and pylor, choledochojejunostomy, gastrojejunostomy and jejunojejunostomy was performed. Primary repair was performed in the remaining patients. In one case with primary repair gastrostomy was performed, while in 9 cases omentoplasty were performed. In the follow-up 12 cases has no problems and doing well. Two patients died. Perforation in duedenum is a rare entity that pediatric surgeons should encounter and keep in mind. Primary repair (duodenoraphy ± omentoplasty) is safe and reliable surgical treatment modality.

BACKGROUND The aim of the present study was to evaluate cases in which intussusception was unsuccessfully treated with pneumatic reduction (PR), and intussusception recurred following PR. METHODS The medical records of 401 patients who presented with intussusception between 2003 and 2014 were retrospectively analyzed. Included were 61 patients, 20 of whom underwent unsuccessful PR (Group 1), and 41 of whom experienced intussusception recurrence following PR (Group 2). Treatment and outcome were summarized. RESULTS In Group 1 (mean age: 14.2 months; range: 2.5 months-6 years) surgery was indicated due to PR failure in 15 patients, and perforation occurred during PR in 5. In these 5 patients, age was under 1 year (range 6-9 months) and mean time to presentation was 3 days (range 2-4). During laparotomy, pathologies were noted: mesenteric lymphadenopathy (LAP) and/or Peyer's patch hyperplasia was observed in 15 cases, Meckel's diverticulum in 5 cases. In Group 2 (mean age: 20 months; range: 3 months-6 years), intussusception developed after successful PR in 41 patients, most frequently within the first 24 hours (21.51%). Of the 41 patients, recurrent intussusception (RI) was treated with PR in 36, and laparotomy in 5. Operative findings were mesenteric LAP in 4 and polyp in 1. CONCLUSION PR is effective for the treatment of intussusception and recurrences. Delayed presentation reduces the success rate. In the event of failure, a lead point is usually encountered at laparotomy.

In this study, we aimed to compare the superiorities of esophageal manometry, vector volume analysis and 24-hour pH meter studies in showing gastroesophageal reflux disease.The files of the patients who presented to pediatric surgery and pediatric gastroenterology outpatient clinics of our hospital with suspicious gastroesophageal reflux disease between 2011 and 2012 and who were investigated were examined and 21 patients whose investigations had been completed were included in the study. The patients were evaluated by treatment method and were divided into three groups as Group 1 who were followed up with medical treatment, Group 2 in whom surgical intervention was performed and Group 3 who were not treated. Chi-square test was used in evaluation of the categorical variables, Kruskal Wallis test was used in comparison of the mean values between the groups and Dunn test was used in subgroup analyses when Kruskal Wallis test was found to be significant. A p value of0.05 was considered statistically significant.Thirteen of 21 patients included in the study were female and eight were male. The mean age of the patients was 5.71 years (one-16 years). In the 24-hour pH monitoring study, the mean reflux index was found to be 48.7% in Group 1, 42.4% in Group 2 and 28.3% in Group 3. In esophageal manometry studies, the pressure difference at lower esophageal sphincter (LES) was found to be 13,4 cm H2O in Group 1, 31.8 cm H2O in Group 2 and 4.3 cmH2O in Group 3. In vector volume analyses, the mean vector volume was calculated to be 96.01 cm(3) in Group 1, 2 398.9 cm(3) in Group 2 and 196.3 cm(3) in Group 3. In the 24-hour pH monitoring study, a statistically significant difference (p0.05) was found in terms of showing reflux, whereas statistical significance could not be shown in terms of need for surgical treatment or need for medical treatment in any other method (p0.05).Twenty-four-hour pH monitoring was found to be efficient in making a diagnosis of gastroesophageal reflux disease, whereas esophageal manometry and vector volume analyses were not found to be efficient.

Here, we report anaesthesia management and the successful use of total intravenous anaesthesia and sugammadex in a patient with Ullrich's disease. Propofol and remifentanyl infusion was used for anaesthesia. After the end of the surgery, when the train-of-four value was 0%, 4 mg kg(-1) sugammadex was administered, and the patient was successfully extubated after 36 s. No adverse effects or safety concerns were observed. In conclusion, we suggest that the use of propofol infusion to avoid the use of inhalation anaesthetics and the use of sugammadex for the reversal of the effects of rocuronium is safe in patients with Ullrich's disease.

Eosinophilic cystitis is an inflammatory condition characterized by eosinophilic infiltration of whole layers of the bladder wall. The condition occurs more commonly in adults. We report a case of eosinophilic cystitis mimicking a bladder tumor in a 5-year-old boy with symptoms of dysuria and urinary incontinence. The diagnosis was confirmed by histopathology and he underwent clinical treatment with trimethoprim-sulfamethoxazole and antihistamine (cetirizine). The symptoms fully resolved in follow up, which is continuing. Although very rare, eosinophilic cystitis should be considered in cases of dysuria and increased bladder wall thickness but no identified urinary tract infection.

We report a 15 years old girl who admitted to our hospital with signs of recurrent torticollis after two failed operations and consistent pain at the side of surgery. The past operations were performed at 1 and 6 years of age and she has been suffering pain from previous incisions with neck movements. At physical examination, the sternocleidomastoid (SCM) muscle behaved like a fibrous band, restricting the neck movements and resulting in pain. The operation was indicated for the fibrotic SCM. At operation two separate incisions were performed on each end of the SCM to remove all of the fibrotic muscle. The histopathological examination demonstrated a traumatic neuroma which respectively correlates with the pain symptoms. The patient discharged on the second postoperative day and physiotherapy was started. The patient is symptom free one year after the surgery. This case demonstrates a rare occurrence of traumatic neuroma after torticollis surgery, which can manifest with pain. Keywords : neuroma, torticollis, trauma

Afibrinogenemia is a rare bleeding disorder which is observed with an incidence of 1:1 000 000. It is an autosomal recessive disease and occurs as a result of mutation in one of the three genes which code the three polypeptide chains of fibrinogen. Basic clinical findings include spontaneous bleeding, bleeding after minor trauma or due to surgery. Splenic rupture in afibrinogenemia has been reported only in 6 cases so far. In this article, we present a 15-year old congenital afibrinogenemia patient with spontaneous splenic rupture.

The spectrum of antibiotics and diseases which are treated with antibiotics are increasing Antibiotics should be used in correct diagnosis indication dose and way of consumption Antibiotics should be started after pending the cultures Empiric choosing of antibiotics could be preferred in emergency and severe conditions Turk Arch Ped 2010; 45: 80th Year: 50 2 Key words: Antibiotics child infection, Antibiyotiklerin sayısı ve kullanım alanları her geçen gün artmaktadır Antibiyotikler doğru tanıyla doğru zamanda doğru kullanım şekli ile ve doğru bir antibiyotik seçimi ile kullanılmalıdır Antibiyotikler mümkünse kültür gönderildikten sonra başlanmalıdır Ampirik antibiyotik kullanımı ciddi ve acil durumlarda tercih edilmelidir Türk Ped Arş 2010; 45: 80 Yıl: 50 2 Anahtar sözcükler: Antibiyotik çocuk enfeksiyon

Amaç: Trakeal veya bronşiyal yabancı cisim aspirasyonu genel anestezi altında bronkoskopi gerektiren çocukluk çağının önemli acillerindendir Bu geriye dönük çalışmada yabancı cisim aspirasyonu nedeniyle uygulanan bronkoskopiler; anestezi yöntemleri riskleri ve işlem sonrası komplikasyonlar açısından değerlendirilmiştir.Gereç ve Yöntem: Çalışmaya Ocak 2006 ve Haziran 2011 yılları arasında çocuk cerrahisi ameliyathanesinde yabancı cisim şüphesiyle bronkoskopi yapılan çocuklar dahil edilmiştir Dört yüz bir çocuk değerlendirmeye alınmış tanı amaçlı bronkoskopi yapılanlar çalışmaya dahil edilmemiştir Tüm bronkoskopilere genel anestezi uygulanmıştır İnhalasyon veya IV indüksiyon ve idamesi kendiliğinden solunum veya kontrollü mekanik ventilasyon şekli şartlara göre seçilmiştir Hastalar klinik fizik muayene radyolojik bulgular anestezi yöntemi ve komplikasyonlar açısından değerlendirilmiştir.Bulgular: Öksürük 82 ve dispne 21 8 trakeobronşiyal yabancı cisimlerin en sık belirtileri olarak tespit edilmiştir En sık rastlanan radyolojik bulgular hava hapsi 57 ve normal akciğer görünümü 21 olarak bulunmuştur Aspire edilen yabancı cisimlerin çoğunun fındık fıstık çekirdek gibi organik maddeler 80 olduğu görülmüş sıklıkla sağ ana bronşa 49 1 ve sol ana bronşa 24 4 yerleşmişlerdir Anestezi sırasında büyük komplikasyon olarak desaturasyon aritmi hiperkarbi ve bronkospazm saptanmıştır.Çıkarımlar: Yabancı cisim inhalasyonunda pek çok sorunla karşılaşılabilir bu hastaların bronkoskopileri anestezi ve cerrahi açıdan doğru zamanda ve en güvenli şekilde deneyimli anestezist ve cerrah tarafından yapılmalıdır., Aim: Glazmann thrombasthenia is a rare autosomal recessive disease characterized by a defect in platelet aggregation Here we report the management of children with Glazmann thrombasthenia followed up at Cerrahpasa Medical Faculty Pediatric Hematology Department Material and Method: The files of nineteen patients 42 girls 58 boys; median age: 10 months were retrospectively reviewed Results: The median age of onset of bleeding symptoms was 9 months 2 weeks 24 months All patients presented with easy bruising and mucosal bleeding Fourteen patients rsquo; parents were consanguineous In 15 patients flow cytometry was performed According to this seven patients had type I six patients had type II and two patients had type III disease Nine patients were treated with thrombocyte transfusion tranexamic acid recombinant active factor VII and fibrin glue as a single or combined therapy; none of them had a major bleeding complication Conclusions: Bleeding control during invasive procedures may be challenging in children with Glazmann thrombasthenia; local treatments desmopressin steroid and antifibrinolytics may be used with success Turk Arch Ped 2012; 47: 104 6