European Journal of Pediatric Surgery Reports最新文献

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Unintentional Suture Fistula Between the Proximal and Distal Esophagus in a Preterm Neonate with Type C Esophageal Atresia 一名患有 C 型食道闭锁的早产新生儿食道近端和远端之间的意外缝合瘘
IF 0.6
European Journal of Pediatric Surgery Reports Pub Date : 2023-12-13 DOI: 10.1055/a-2227-6252
Julia Emma Menso, Maud A Reijntjes, Carlijn Mussies, M. V. van Wijk, Sander Zwaveling
{"title":"Unintentional Suture Fistula Between the Proximal and Distal Esophagus in a Preterm Neonate with Type C Esophageal Atresia","authors":"Julia Emma Menso, Maud A Reijntjes, Carlijn Mussies, M. V. van Wijk, Sander Zwaveling","doi":"10.1055/a-2227-6252","DOIUrl":"https://doi.org/10.1055/a-2227-6252","url":null,"abstract":"We present the case of a patient with Gross type C esophageal atresia in a preterm neonate (gestational age 31+1) with a birth-weight of 1470 grams. The fistula was released via a thoracotomy, but no primary anastomosis could be made, due to an unexpected long gap. The distal esophagus was closed and approximated to the blind pouch using traction sutures until an adjacent position was reached. A gastrostomy was created for enteral feeding. Although a second attempt to make an anastomosis was unsuccessful, the patient unexpectedly developed a suture fistula six weeks after the first procedure, enabling feeding via a nasogastric tube. Over time, six dilations were necessary. Full enteral feeding was achieved at the age of six months. Our case confirms sparse reports that deliberately creating a suture fistula may be a solution in esophageal atresia patients when an unexpected long gap prohibits a primary anastomosis.","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":"4 1","pages":""},"PeriodicalIF":0.6,"publicationDate":"2023-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139004687","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Missed proximal tracheoesophageal fistula (TEF) in a neonate with Type D esophageal atresia 一名患有 D 型食管闭锁的新生儿被漏诊的近端气管食管瘘(TEF)
IF 0.6
European Journal of Pediatric Surgery Reports Pub Date : 2023-12-13 DOI: 10.1055/a-2227-6389
Julia Emma Menso, Maud A Reijntjes, M. Oomen, Rico Npm Rinkel, Suzanne WJ Terheggen - Lagro, Ramon Gorter
{"title":"Missed proximal tracheoesophageal fistula (TEF) in a neonate with Type D esophageal atresia","authors":"Julia Emma Menso, Maud A Reijntjes, M. Oomen, Rico Npm Rinkel, Suzanne WJ Terheggen - Lagro, Ramon Gorter","doi":"10.1055/a-2227-6389","DOIUrl":"https://doi.org/10.1055/a-2227-6389","url":null,"abstract":"We present the case of a patient with the rare type D esophageal atresia (EA), diagnosed after correction of an initially as type C diagnosed EA. Routine postoperative contrast esophagogram showed a missed proximal tracheoesophageal fistula. This case report illustrates the potential difficulties to diagnose type D EA.","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":"22 4","pages":""},"PeriodicalIF":0.6,"publicationDate":"2023-12-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139004805","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Massive chylous ascites in nine-year-old with malrotation - a case report 九岁儿童腹腔畸形并发大量乳糜腹水--病例报告
IF 0.6
European Journal of Pediatric Surgery Reports Pub Date : 2023-12-04 DOI: 10.1055/a-2221-9682
Hans Winberg, Pär Gerwins, Kristine Hagelsteen
{"title":"Massive chylous ascites in nine-year-old with malrotation - a case report","authors":"Hans Winberg, Pär Gerwins, Kristine Hagelsteen","doi":"10.1055/a-2221-9682","DOIUrl":"https://doi.org/10.1055/a-2221-9682","url":null,"abstract":"Malrotation leading to massive chylous ascites is rare. A nine-year-old girl was investigated for slowly increasing abdominal distension under a year. She had no vomiting, weight loss or pain, but was bothered in social situations. Medical investigations, including ultrasound and CT scans, revealed massive ascites. Laparocentesis yielded milk colored fluid, confirmed as lymph through laboratory analysis. A complete blood count, liver function and hematologic parameters, chyle cytology, bacterial cultures and PCR for tuberculosis were all within normal limits. \u0000She was referred to a tertiary center for vascular anomalies. A dynamic contrast enhanced MR lymphangiography showed normal lymphatic anatomy without leakage or flow obstruction. A whole-body MRI revealed a central mesenteric rotation. \u0000She was referred to a tertiary center for pediatric surgery, where a laparoscopic Ladd’s procedure was performed using a new 5 mm pediatric sealing device, along with an appendectomy using a 5 mm stapler. To derotate the bowel, fenestrations were created in compartments containing a substantial amount of chyle and ascites, resulting in the drainage of 2.4 liters of fluid. She was discharged the day after surgery and has been in good health for one year. We present a video illustrating the Ladd’s procedure steps in this patient.","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":"40 16","pages":""},"PeriodicalIF":0.6,"publicationDate":"2023-12-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138602671","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Neonatal sacrococcygeal fetiform teratoma containing bowel: A case report 新生儿骶尾骨畸形畸胎瘤含肠1例
European Journal of Pediatric Surgery Reports Pub Date : 2023-11-08 DOI: 10.1055/a-2206-4825
Ashley Toms, Giulia Brisighelli, Tarlia Rasa Govender, Derek Harrison
{"title":"Neonatal sacrococcygeal fetiform teratoma containing bowel: A case report","authors":"Ashley Toms, Giulia Brisighelli, Tarlia Rasa Govender, Derek Harrison","doi":"10.1055/a-2206-4825","DOIUrl":"https://doi.org/10.1055/a-2206-4825","url":null,"abstract":"A fetiform sacrococcygeal teratoma (homunculus) is a highly differentiated subgroup of mature cystic teratoma that resembles a malformed fetus. These tumours originate at the base of the coccyx and may vary in their intrapelvic and extrapelvic extent and location. It is important to differentiate this anomaly from fetus-in-fetu which have a higher degree of structural organisation. A 5-day old neonate presented with a type II sacrococcygeal fetiform teratoma. The mass contained both cystic and solid components. Upon surgical excision and coccygectomy, fully formed bowel was found inside the mass, as well as bones and other well- defined structures. The tumour was confirmed to be fully excised and no malignant or immature features were found on histopathological examination. The patient was last seen growing well with an AFP of 3.5 μg/l, 14 months after resection.","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":"6 10","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135391768","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Posterior Sinuplasty: a new strategy for managing hydrocolpos in cloaca – case series 后窦成形术:治疗泄殖腔积液的新策略
European Journal of Pediatric Surgery Reports Pub Date : 2023-11-06 DOI: 10.1055/a-2204-8629
Amr AbdelHamid AbouZeid, Ahmed Saad Abdelmoniem, mohamed abdelrahman, Mohamed Ahmed Negm, Shaimaa Abdelsattar Mohammad
{"title":"Posterior Sinuplasty: a new strategy for managing hydrocolpos in cloaca – case series","authors":"Amr AbdelHamid AbouZeid, Ahmed Saad Abdelmoniem, mohamed abdelrahman, Mohamed Ahmed Negm, Shaimaa Abdelsattar Mohammad","doi":"10.1055/a-2204-8629","DOIUrl":"https://doi.org/10.1055/a-2204-8629","url":null,"abstract":"We present a simple surgical technique aiming to improve urine outflow through the common urogenital sinus in cloaca and facilitate drainage of existing hydrocolpos. The study included three cases of cloaca with associated hydrocolpos that were operated during the period 2022 through 2023. Surgical technique: The patient is placed in the prone position for a standard Posterior Sagittal AnoRectoPlasty (PSARP). The distal rectal fistula is severed flush with the vagina/sinus leaving an open defect in the posterior wall of the vagina/sinus. The defect is then widened distally via a vertical incision (about 1cm) through the posterior wall of the common urogenital sinus towards but not reaching the perineum. This vertical defect is then closed horizontally displacing the posterior vaginal wall downwards towards the perineum (Posterior sinuplasty). The postoperative recovery was uneventful in the three cases. Adequate drainage of hydrocolpos was confirmed by imaging at follow up, as well as improvement of upper urinary tract dilatation. Conclusion: In selected cases of cloaca, posterior sinuplasty is a simple procedure that can be applied during anorectoplasty to provide effective drainage of associated hydrocolpos.","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":"9 6","pages":"0"},"PeriodicalIF":0.0,"publicationDate":"2023-11-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135680019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Endobronchial Inflammatory Myofibroblastic Tumor in a 3-Year-Old Child. 3岁儿童支气管内炎性肌成纤维细胞瘤1例。
IF 0.6
European Journal of Pediatric Surgery Reports Pub Date : 2023-03-09 eCollection Date: 2023-01-01 DOI: 10.1055/s-0043-1764289
Riccardo Guanà, Andrea Carpino, Marta Miglietta, Elisa Zambaiti, Alessia Cerrina, Luca Lonati, Francesco Guerrera, Stefano Vallero, Salvatore Garofalo, Marco Bardessono, Francesca Maletta, Steffi Shilly, Fabrizio Gennari
{"title":"Endobronchial Inflammatory Myofibroblastic Tumor in a 3-Year-Old Child.","authors":"Riccardo Guanà, Andrea Carpino, Marta Miglietta, Elisa Zambaiti, Alessia Cerrina, Luca Lonati, Francesco Guerrera, Stefano Vallero, Salvatore Garofalo, Marco Bardessono, Francesca Maletta, Steffi Shilly, Fabrizio Gennari","doi":"10.1055/s-0043-1764289","DOIUrl":"10.1055/s-0043-1764289","url":null,"abstract":"<p><p>Inflammatory myofibroblastic tumor (IMT) is a mesenchymal tumor that can occur at any age. However, it is primarily seen in children, with the most common site being in the lung parenchyma, usually present with rare endobronchial lesions. This case reports the incidence in a 3-year-old girl diagnosed with pericardiac pneumonia treated with antibiotics with no clinical improvement. A chest computed tomography (CT) scan identified a 1.5-cm lesion in the left main bronchus. Bronchoscopy revealed complete obstruction of the left main stem bronchus. A left posterolateral thoracotomy was performed. Additionally, a left sleeve upper bronchial resection was conducted under fibroendoscopic control. Definitive histology confirmed IMT. After 2 years of endoscopic follow-up, there is no evidence of recurrence.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":"11 1","pages":"e5-e9"},"PeriodicalIF":0.6,"publicationDate":"2023-03-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9998215/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9155688","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Endoscopic Treatment for Nonhypertrophic Idiopathic Pyloric Stenosis in an Adolescent Patient. 非肥厚性特发性幽门狭窄青少年患者的内镜治疗。
IF 0.6
European Journal of Pediatric Surgery Reports Pub Date : 2023-01-01 DOI: 10.1055/s-0043-57040
Carlo Maria Ferlini, Elena De Lorenzi, Cristina Belgiovine, Emanuele Cereda, Silvia Maria Elena Caimmi, Alessandro Raffaele
{"title":"Endoscopic Treatment for Nonhypertrophic Idiopathic Pyloric Stenosis in an Adolescent Patient.","authors":"Carlo Maria Ferlini,&nbsp;Elena De Lorenzi,&nbsp;Cristina Belgiovine,&nbsp;Emanuele Cereda,&nbsp;Silvia Maria Elena Caimmi,&nbsp;Alessandro Raffaele","doi":"10.1055/s-0043-57040","DOIUrl":"https://doi.org/10.1055/s-0043-57040","url":null,"abstract":"<p><p>Nonhypertrophic idiopathic pyloric stenosis (NHIPS) is a rare occurrence in children. It could be related to peptic ulcers, but a definitive cause is yet to be found. Treatment is a matter of debate, ranging from medical to surgical. We report the case of a 15-year-old boy suffering postprandial vomiting and weight loss in the previous 3 months. NHIPS was diagnosed and successfully treated with several sessions of endoscopic pyloric dilation and jejunal feeding. In association with a multidisciplinary approach, endoscopic dilation should be considered as a first-line treatment to avoid surgery.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":"11 1","pages":"e25-e28"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10156441/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9436026","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Upper Ureteral Reconstruction with a Tapered Descending Colon after Failed Pyeloplasties in a 13-Year-Old Boy. 13岁男孩肾盂成形术失败后输尿管上段以锥形降结肠重建一例。
IF 0.6
European Journal of Pediatric Surgery Reports Pub Date : 2023-01-01 DOI: 10.1055/a-2035-4637
Hideaki Nakajima, Hiroyuki Koga, Seitaro Kosaka, Mao Ikari, Geoffrey J Lane, Atsuyuki Yamataka
{"title":"Upper Ureteral Reconstruction with a Tapered Descending Colon after Failed Pyeloplasties in a 13-Year-Old Boy.","authors":"Hideaki Nakajima,&nbsp;Hiroyuki Koga,&nbsp;Seitaro Kosaka,&nbsp;Mao Ikari,&nbsp;Geoffrey J Lane,&nbsp;Atsuyuki Yamataka","doi":"10.1055/a-2035-4637","DOIUrl":"https://doi.org/10.1055/a-2035-4637","url":null,"abstract":"<p><p>An 11-year-old boy was referred for further management of a 6-cm-long grossly stenosed ureter following two failed left ureteropelvic junction (UPJ) obstruction repairs elsewhere. A tapered segment of the descending colon (TDC) was used successfully for ureteral reconstruction. The UPJ was exposed through a left flank incision. The stenosed segment was excised; both ends appeared severely inflamed and thickened. Tissue interposition was required and ureteroplasty with a TDC was performed by incising the peritoneum adjacent to the excised ureter to mobilize the descending colon to the retroperitoneal space. To prepare the TDC, an 8-cm segment of the colon with intact blood vessels was isolated, tapered, and sutured into a funnel shape using a 14-Fr catheter as a temporary stent. After colocolostomy, the colon was returned to the abdominal cavity, the peritoneum was closed carefully to prevent vascular compromise, and the TDC was anastomosed to the ureter and renal calyx with interrupted absorbable sutures. A double J stent (DJS) and percutaneous nephrostomy tube were placed. Postoperative recovery was uneventful. The DJS was removed on day 50 after confirming smooth urine flow through both the ureter-TDC and calyx-TDC anastomoses. Diuretic renography performed 68 days postoperatively was unobstructed. The patient is currently well after 12 months follow-up. This would appear to be the first report of a TDC being used to create a funnel-shaped segment to reconstruct a long, grossly stenosed ureter. The TDC is simpler than the re-tubularizing colon but requires monitoring for postoperative mucus-related complications and malignant transformation.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":"11 1","pages":"e10-e14"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10085642/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9304516","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Congenital Internal Jugular Phlebectasia: An Anomaly Still Poorly Recognized. 先天性颈内静脉扩张:一种仍未被充分认识的异常。
IF 0.6
European Journal of Pediatric Surgery Reports Pub Date : 2023-01-01 DOI: 10.1055/a-2130-3269
Alessandro Raffaele, Marta Gazzaneo, Piero Romano, Maria Sole Prevedoni Gorone, Luigi Avolio
{"title":"Congenital Internal Jugular Phlebectasia: An Anomaly Still Poorly Recognized.","authors":"Alessandro Raffaele,&nbsp;Marta Gazzaneo,&nbsp;Piero Romano,&nbsp;Maria Sole Prevedoni Gorone,&nbsp;Luigi Avolio","doi":"10.1055/a-2130-3269","DOIUrl":"https://doi.org/10.1055/a-2130-3269","url":null,"abstract":"<p><p>Congenital internal jugular phlebectasia (CIJP) is a rare condition characterized by congenital dilatation of the vein without tortuosity that becomes more evident during straining as a lateral neck mass. CIJP often remains undiagnosed from a few months to several years after the onset of the swelling. It is frequently asymptomatic although symptomatic cases have been occasionally reported. We present the case of a healthy 7-year-old boy with a lateral neck mass, triggered by the Valsalva maneuver. Neck ultrasound (US) showed right internal jugular axial ectasia, increasing during the Valsalva maneuver; contrast computed tomography (CT) scan confirmed a fusiform dilatation of the right internal jugular vein. Due to the lack of symptoms, we treated our patient conservatively. At 5 years of follow-up, the patient is still asymptomatic, with no evidence of complications or thrombosis. Due to its self-limiting nature, treatment for asymptomatic cases of CIJP should be conservative, providing a follow-up with both clinical and US annual evaluations.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":"11 1","pages":"e40-e43"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10471426/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10142883","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Successful Sirolimus Treatment for Recurrent Pericardial Effusion in a Large Cervicomediastinal Provisionally Unclassified Vascular Anomaly: A Case Report. 西罗莫司成功治疗颈纵隔大血管异常复发性心包积液1例。
IF 0.6
European Journal of Pediatric Surgery Reports Pub Date : 2023-01-01 DOI: 10.1055/a-2057-7177
Julio César Moreno-Alfonso, María San Basilio Berenguer, María Del Carmen Sarmiento Caldas, Jesús González Cayón, Santiago de la Puente, Paloma Triana, Juan Carlos López-Gutiérrez
{"title":"Successful Sirolimus Treatment for Recurrent Pericardial Effusion in a Large Cervicomediastinal Provisionally Unclassified Vascular Anomaly: A Case Report.","authors":"Julio César Moreno-Alfonso,&nbsp;María San Basilio Berenguer,&nbsp;María Del Carmen Sarmiento Caldas,&nbsp;Jesús González Cayón,&nbsp;Santiago de la Puente,&nbsp;Paloma Triana,&nbsp;Juan Carlos López-Gutiérrez","doi":"10.1055/a-2057-7177","DOIUrl":"https://doi.org/10.1055/a-2057-7177","url":null,"abstract":"<p><p>Provisionally unclassified vascular anomalies (PUVA) are a group of diseases with unique characteristics that make them unclassifiable within vascular tumors or malformations. We describe a PUVA as the cause of recurrent pericardial effusion and its response to sirolimus. A 6-year-old girl was referred with a cervicothoracic vascular anomaly, a violaceous, and irregular lesion in the neck and upper chest, diagnosed as \"hemangioma\". She had pericardial effusion at the neonatal age that required pericardiocentesis, propranolol, and corticosteroids. She remained stable for 5 years, when she presented with a severe pericardial effusion. A magnetic resonance visualized a diffuse vascular image in the cervical and thoracic region with mediastinal extension. The pathological study showed a vascular proliferation in the dermis and hypodermis with positive staining for Wilms' Tumor 1 Protein (WT1) and negative for Glut-1. Genetic testing found a variant in <i>GNA14</i> , for which the diagnosis of PUVA was established. When a pericardial drain was placed without response, treatment with sirolimus was started with resolution of the effusion. Sixteen months later, the malformation is stable and there has been no recurrence of pericardial effusion. In a significant group of patients, definitive diagnosis is not possible despite pathological and genetic analysis. Mammalian target of rapamycin inhibitors may become a therapeutic option if symptoms are severe enough, with a low rate of reported side effects.</p>","PeriodicalId":43204,"journal":{"name":"European Journal of Pediatric Surgery Reports","volume":"11 1","pages":"e20-e24"},"PeriodicalIF":0.6,"publicationDate":"2023-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10191730/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9868952","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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