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    Hedgehog Signal Defect Leading to Familial Exudative Vitreoretinopathy-Like Disease and Gastrointestinal Malformation
    (2022) Keskek, Nedime Sahinoglu; Akkoyun, Imren; Temiz, Abdulkerim; Kutuk, Ozgur; https://orcid.org/0000-0001-8544-103X; https://orcid.org/0000-0002-2860-7424; https://orcid.org/0000-0001-9854-7220; 35770050; T-4258-2017; AAK-7713-2021
    Objectives: The aim of the study was to present a new generic association presenting with gastrointestinal tract malformations (GTMs) and familial exudative vitreoretinopathy (FEVR)-like disease and review the genetics of Hedgehog signaling. Materials and Methods: Three neonates were diagnosed with FEVR-like retinal vascular disease upon routine ophthalmological examination during hospitalization in the neonatal surgical intensive care unit for GTMs. Generic analysis of the neonates was performed. Results: Gestational age of the neonates was 39, 38, and 39 weeks and birth weights were 3,500, 3,600, and 3,300 grams, respectively. All six eyes of the three infants were treated by laser photocoagulation. Recurrence was not seen in any of the eyes. Genetical analysis of all the neonates diagnosed with FEVR-like disease revealed defects in the Hedgehog pathway. Conclusion: FEVR is a genetically well-defined retinal vascular disease. The current study is the first to show an association between FEVR-like retinal vascular disease and GTMs. This study demonstrates the importance of the Hedgehog pathway in retinal vascular and gut development.
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    Pancreatic Tumors in Children
    (2021) Gezer, Hasan Ozkan; Temiz, Abdulkerim; Ezer, Semire; Yazici, Nalan; Demir, Senay; Hasbay, Bermal; Oguzkurt, Pelin; 0000-0002-4635-2613; 0000-0002-4209-9075; 0000-0001-6702-7265; A-4719-2018; AAJ-9529-2021; AAM-5138-2021; AAK-9310-2021
    Objective: Pancreatic rumors in children are exceedingly rare and hence present diagnostic and therapeutic challenges to pediatric surgeons. In this study, we aimed to present our experiences and treatment outcomes related to these rare tumors. Methods: The clinical data, laboratory investigations, radiological imaging, and the pathology and surgical details of patients with pancreatic tumors who were diagnosed between 2005 and 2019 were retrospectively reviewed. Results: A total of 9 patients (5 men) were included in the study. The most common symptom at the time of presentation was vague abdominal pain. A pancreatic rumor was detected incidentally in 4 patients. All tumors were non-functional primary rumors. Histopathological diagnosis of these tumors were solid-pseudopapillary tumors (n=3), congenital pancreatic cysts (n=3), pancreatoblastoma (n=1), rhabdomyosarcoma (n=1), and an undifferentiated carcinoma (n=1). In addition, 8 patients were treated surgically (through tumor excision, central pancreatectomy + distal pancreaticojejunostomy, distal pancreatectomy, and cystogastrostomy). Two deaths from tumor dissemination were recorded. The patients were followed-up at a mean duration of 72 months (range: 6-120 months). Conclusion: Pediatric pancreatic tumors arc rare and are usually benign in nature. They present symptoms that are often nonspecific. In non-metastatic cases, surgical tumor removal is the preferred method for the treatment. For most tumors, surgical resection is the optimal treatment that may be successfully performed with low morbidity rate when the lesion is either in the body or in the tail of the pancreas. The long-term outcomes with this approach are generally good.
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    Ingested foreign bodies in children: Do they really pass spontaneously from the gastrointestinal tract? A single-centre experience with 1000 cases
    (2020) Gezer, Hasan Ozkan; Ezer, Semire Serin; Temiz, Abdulkerim; Ince, Emine; Hicsonmez, Akgun; 0000-0002-9597-3264; 0000-0002-4635-2613; 0000-0001-8789-6003; 32185763; AAJ-9529-2021; J-3197-2013; A-4719-2018
    BACKGROUND: Foreign body (FB) ingestion is frequently encountered in all departments that treat children. FB may bring about significant anxiety for parents and physicians. The present study aims to determine the appropriate approach for FB ingestion in children. METHODS: The records of 1000 children with a history of FB ingestion between the years 2005 and 2017 were reviewed retrospectively in this study. RESULTS: Of 1000 children, 53.8% were male. The most common types of FBs were coins (35%). X-ray was negative in 49% of the patients, and 86% of these patients received no intervention. Of the 504 (51%) X-ray-positive patients, the oesophagus (68%) was the most common location. Life-threatening complications were tracheo-oesophageal fistula (I), Meckel's diverticulum perforation (I), and perforation due to rigid endoscopy (I). CONCLUSION: We demonstrated that coins, which are the most commonly ingested FBs, have various types and sizes according to their countries of origin, and this affects spontaneous passage. We found that only 48% (quite low compared to the literature) of the coins passed spontaneously. In asymptomatic patients with a gastric button battery, we suggest a "watchful waiting" approach. The patients should be observed and managed at home. In our study, we found that 85% of the button batteries that reached the stomach passed spontaneously.
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    Gastroschisis Treatment: Evaluation of Surgical Techniques and Results
    (2020) Ince, Emine; Ezer, Semire Serin; Temiz, Abdulkerim; Gezer, Hasan Ozkan; Hicsonmez, Akgun; A-4719-2018; AAJ-9529-2021
    Objective: Recently, achieving good cosmetic results for patients with gastroschisis has gained popularity, as since the visceral organs can be safely positioned into the abdominal cavity. We aimed to evaluate patient outcomes while focusing on the cosmetic results to contribute to the limited amount of data on this subject in the literature. Methods: From January 2005 to May 2018, patients operated on for gastroschisis in a single institution were evaluated retrospectively. Results: Twenty-two patients with gastroschisis were treated (10 females/12 males). The mean gestational age was 38.2 +/- 2.1 weeks. The mean birth weight was 2256.9 +/- 418.2 g. Eleven patients were diagnosed prenatally. Five patients had complex gastroschisis. Others presented with necrosis (n=3), perforation (n=3), volvulus (n=1) and jejunal atresia (n=1). Primary closure (n=16) and staged closure (n=6) were performed. Patients needed mechanical ventilation and total parenteral nutrition for 3.7 +/- 1.0 days and 24.3 +/- 9.7 days, respectively. Enteral feeding was started at 15.9 +/- 10.5 days. The duration of hospitalisation was 36.7 +/- 13.3 days. Three patients died from sepsis and multi-organ failure. Conclusion: We believe that performing appropriate gastroschisis surgery at the same hospital without transferring to another centre is the main factor in achieving a successful outcome. Good cosmetic results and visceral function were achieved in this study in 86.3% patients.
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    Post-necrotizing Enterocolitis Stricture: Misdiagnosis of this Complication Results in Greater Infant Mortality
    (2019) Ince, Emine; Ezer, Semire Serin; Temiz, Abdulkerim; Gezer, Hasan Ozkan; Hicsonmez, Akgun; J-3197-2013
    Objective: Intestinal stricture following necrotizing enterocolitis (NEC) is often misdiagnosed as recurrent functional constipation, enteritis, and malnutrition, and it increases the rates of morbidity and mortality in infants. Although a number of studies have focused on the potential etiologic factors leading to NEC, the information regarding the occurrence and diagnosis of post-NEC strictures is limited. The aim of this study was to evaluate the clinical presentation and diagnostic and surgical methods to treat NEC. Materials and Methods: The medical records of infants who had undergone surgery for post-NEC strictures between January 2005 and September 2018 were evaluated retrospectively in a single institution. Results: This study included 38 infants (20 males, 18 females) with post-NEC stricture. Their histories revealed that they had been treated medically (20 of 38) or surgically (18 of 38) for NEC. Symptoms typical of intestinal obstruction (vomiting, abdominal distension, constipation, growth retardation, etc.) were present in the medically treated patients. The average time of onset of symptoms after the acute episode of NEC was 1.64 +/- 0.78 months. Contrast studies revealed strictures in the small intestine in 13 (65%) medically treated patients, while 13 (72.2%) surgically treated patients had strictures in the colon. Additionally, 2 of surgically treated patients presented with ileocolic fistulae. In 11 of 38 (28.9%) patients, the contrast studies were false-negative. Conclusion: Post-NEC strictures may present with vague nutritional problems, causing the diagnosis to often be missed, which leads to high rates of morbidity and mortality in infants. Colon enemas, distal loopograms, and small bowel passage radiograms are useful in making a diagnosis, but a careful examination of the intestines for the presence of any other strictures should be done during the surgery.
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    Boix-Ochoa (Partial Fundoplication) Treats Reflux, Even in Neurologically Impaired Patients. Can it Take the Title of "Gold Standard" from Total Fundoplication?
    (2019) Gezer, Hasan Ozkan; Ezer, Semire Serin; Temiz, Abdulkerim; Ince, Emine; Hicsonmez, Akgun; 0000-0002-4635-2613; 30887292; J-3197-2013
    Background In 4-5% of cases of gastroesophageal reflux disease (GERD), surgical treatment is required. The aim of the study was to evaluate the success of Boix-Ochoa antireflux surgery, which is considered more physiologic with a higher failure rate (need for reoperation) than Nissen fundoplication, which is believed to be the gold standard operation. Method In the 13 years from 2005 to 2018, the medical records of all children who underwent Boix-Ochoa in a single institution by pediatric surgeons were reviewed retrospectively. Results A total of 133 fundoplications were performed, of which patients were divided into four groups: neurologically impaired, structurally impaired, neurologically and structurally impaired, and neurologically and structurally normal; there were 64, 8, 34, and 27 patients in each group, respectively. Structural impairments included hiatal hernia and esophagus atresia, having previously had a gastrostomy and esophageal stenosis. The most common short-term complication was distal esophageal stenosis (13%), which caused vomiting and dysphagia, and was treated by dilatations. There were six (4.5%) recurrences of GER, one in the neurologically and structurally impaired group with a hiatal hernia and five in the structurally impaired group (three esophagus atresias, two caustic esophageal strictures). The mean follow-up period was 5.27 +/- 3.43 years. Neurological impairment did not affect the success rate. Conclusion Although there has not any literature demonstrating significant benefits of one procedure, we detected with this largest study in the pediatric literature about Boix-Ochoa fundoplication (more physiologic and easily performed) that it was successful (95%) in protecting reflux even in neurologically impaired patients (98%). We consider Boix-Ochoa (partial fundoplication) to be an alternative method to Nissen (complete fundoplication), and it can be done safely with a high success rate.
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    Spleen Salvaging Treatment Approaches in Non-parasitic Splenic Cysts in Childhood
    (2016) Gezer, Hasan Ozkan; Oguzkurt, Pelin; Temiz, Abdulkerim; Ince, Emine; Ezer, Semire Serin; Kocer, Nazim Emrah; Demir, Senay; Hicsonmez, Akgün; 27574347
    The aim of this study was to evaluate our experience with primary non-parasitic splenic cysts (NPSC) which are relatively rare in children and consist almost exclusively of single case reports or small case series in the literature. The medical records of all patients who presented to our clinic with NPSC between 2005 and 2015 were evaluated retrospectively. There were 22 children whose ages ranged from 2 months to 14 years (mean 9.2 +/- 4.7 years). The size of the cysts was in the range of 5 to 200 mm (mean 55.4 +/- 48.2 mm). Ten patients underwent surgery for splenic cysts. Partial splenectomy (n = 2), total cyst excision (either open n = 4 or laparoscopically n = 1), and total splenectomy (n = 3) were performed. The non-operated patients were asymptomatic and followed with ultrasound (US). The follow-up period in non-operated patients ranged from 6 months to 5 years (mean 2.27 +/- 1.29 years). Complete regression was observed in four (33 %) non-operated patients. The regressed cyst measurements were 10, 16, 30, and 40 mm, respectively. Approximately half of the NPSC is diagnosed incidentally. Small (< 5 cm) asymptomatic cysts should be under regular follow-up with US/physical examination for regression. If surgery is required, we prefer open cyst excision as it gives excellent results and preserves splenic immune function.
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    Huge Neck Masses Causing Respiratory Distress in Neonates: Two Cases of Congenital Cervical Teratoma
    (2016) Gezer, Hasan Ozkan; Oguzkurt, Pelin; Temiz, Abdulkerim; Bolat, Filiz Aka; Hicsonmez, Akgun; 0000-0002-4635-2613; 0000-0001-8789-6003; 25176284; J-3197-2013; A-4719-2018
    Congenital cervical teratomas are rare and usually large enough to cause respiratory distress in the neonatal period. We present two cases of congenital huge cystic neck masses in which distinguishing cervical cystic hygroma and congenital cystic teratoma was not possible through radiologic imaging techniques. Experience with the first case, which was initially diagnosed and treated as cystic hygroma by injection sclerotherapy, led to early suspicion and surgery in the second case. The masses were excised completely and histopathologic diagnoses were congenital teratoma in both patients. Our aim is to review congenital huge neck masses causing respiratory distress in early neonatal life to highlight this dilemma briefly with these interesting cases. Copyright (C) 2014, Taiwan Pediatric Association. Published by Elsevier Taiwan LLC. All rights reserved.
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    Approaches to Female Congenital Genital Tract Anomalies and Complications
    (2017) Ince, Emine; Oguzkure, Pelin; Ezer, Semire Serin; Temiz, Abdulkerim; Gezer, Hasan Ozkan; Demir, Senay; Hicsonmez, Akgun; 0000-0002-4209-9075; 0000-0002-9597-3264; 0000-0001-8789-6003; 0000-0002-4635-2613; A-4719-2018; AAJ-9529-2021; J-3197-2013; AAK-9310-2021
    Objective: Female congenital genital tract anomalies may appear with quite confusing and deceptive complications. This study aims to evaluate the difficulties in diagnosis and treatment of female congenital genital tract anomalies that frequently present with complications. Summary: During a 10-year period, we evaluated 20 female patients with congenital genital tract anomalies aged between 3 days and 16 years. All patients were retrospectively analyzed in terms of the results of diagnostic studies, surgical intervention, and treatment. Methods: Ultrasonography and magnetic resonance imaging revealed hydromucocolpos or hematocolpometra, imperforate hymen, distal vaginal atresia, didelphys uterus, an obstructed right hemivagina, uterovaginal atresia, a unicornuate uterus with a noncommunicating rudimentary horn, a vesicovaginal fistula, a utero-rectal fistula, intraabdominal collection, and a vaginal calculus. Results: Two patients had Mayer-Rokitansky-Kuster-Hauser syndrome and 6 patients had obstructed hemivagina and ipsilateral renal anomaly syndrome. Definitive surgical interventions were hymenotomy, vaginal pull-through, vaginovaginostomy, and vesico-vaginal fistula repair using a transvesical approach. In conclusion, female congenital genital tract anomalies may appear with a wide range of complications. Conclusions: There is a potential to do significant harm, if the patient's anatomic problems are not understood using detailed imaging. Revealing the anatomy completely and defining the complications that have already developed are critical to tailor the optimal treatment strategies and surgical approaches.
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    Endovascular Diagnosis and Successful Treatment of Massive Gastrointestinal Hemorrhage in Children
    (2018) Temiz, Abdulkerim; Gedikoglu, Murat; Ezer, Semire Serin; Oguzkurt, Pelin; Hicsonmez, Akgun; 29666037