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Myeloid sarcomas: Experience from a pediatric oncology referral center. 髓系肉瘤:来自儿科肿瘤转诊中心的经验。
M Banzo Navascués, M Martínez Díaz, J Cortés Sáez, J Mira Abenza, A Marco Macián

Objective: To analyze the experience in the management of myeloid sarcomas at a pediatric oncology referral center.

Material and methods: A descriptive, retrospective study of patients under 18 years of age diagnosed with myeloid sarcoma between 2010 and 2024. Demographic variables, underlying disease, tumor location, tumor size, treatment, and clinical outcome were collected. The analysis was performed using IBM® SPSS Statistics 30.0.

Results: Seventeen myeloid sarcomas were identified in 14 patients, 13 (76.5%) of whom were male. The median age at diagnosis was 4.33 years (range: 1 month-14.5 years). In 10 cases (58.8%), the sarcoma was the first manifestation of an underlying hematologic malignancy. Biopsy was performed in 12 cases (70.6%). The most frequent locations were soft tissues (47.1%) and bone (23.5%). The mean tumor volume was 45.2 cm3. Acute myeloid leukemia was the most common underlying neoplasm (n= 14), followed by B-cell acute lymphoblastic leukemia (n= 3). Eight myeloid sarcomas (47.1%) resolved after a first cycle of chemotherapy, 6 (35.3%) required additional cycles, 2 (11.8%) received local treatment, and in 1 case (5.9%) resolution was not achieved.

Conclusions: Myeloid sarcoma represents an uncommon extramedullary manifestation. Early diagnosis, based on a high index of suspicion and appropriate clinical and radiological characterization, is crucial to establish an effective therapeutic approach and improve prognosis.

目的:分析某小儿肿瘤转诊中心髓系肉瘤的治疗经验。材料和方法:对2010年至2024年间诊断为髓系肉瘤的18岁以下患者进行描述性回顾性研究。收集人口统计学变量、基础疾病、肿瘤位置、肿瘤大小、治疗和临床结果。采用IBM®SPSS Statistics 30.0进行分析。结果:14例患者中发现17例髓系肉瘤,其中男性13例(76.5%)。诊断时的中位年龄为4.33岁(范围:1个月-14.5岁)。在10例(58.8%)中,肉瘤是潜在的血液系统恶性肿瘤的首发表现。活检12例(70.6%)。最常见的部位是软组织(47.1%)和骨骼(23.5%)。平均肿瘤体积为45.2 cm3。急性髓系白血病是最常见的基础肿瘤(n= 14),其次是b细胞急性淋巴母细胞白血病(n= 3)。8例(47.1%)骨髓肉瘤在第一个化疗周期后消退,6例(35.3%)需要额外的化疗周期,2例(11.8%)接受局部治疗,1例(5.9%)未实现消退。结论:髓系肉瘤是一种罕见的髓外表现。早期诊断,基于高怀疑指数和适当的临床和放射学特征,对于建立有效的治疗方法和改善预后至关重要。
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引用次数: 0
The role of contrast-enhanced ultrasound in the diagnosis of gastroesophageal reflux. 超声造影在胃食管反流诊断中的作用。
C Parrondo Muiños, T Moratalla Jareño, M S Fernández Córdoba, L C Fernández Masaguer

Objective: To assess the use of contrast-enhanced ultrasonography in the diagnosis of gastroesophageal reflux and hiatal hernia, avoiding ionizing radiation.

Material and methods: Seven contrast-enhanced esophagogastric ultrasound examinations were performed in children between 5 months and 3 years of age, 5 to evaluate gastroesophageal reflux and hiatal hernia, and 2 for gastroesophageal reflux. An ultrasound machine with specific software for contrast visualization, a multi-frequency probe, and sonographic contrast agent were used. Findings were correlated with upper gastrointestinal series in 4 cases.

Results: Of the 7 patients, 4 were awaiting percutaneous endoscopic gastrostomy (2 with a nasogastric tube and 2 with vomiting, 1 of them also with a nasogastric tube), and the remaining three had undergone surgery: 1 for hypertrophic pyloric stenosis, another for esophageal atresia, both also with vomiting; and the last was a patient with percutaneous endoscopic gastrostomy and suspected gastroesophageal reflux disease. The following were observed: 2 cases without gastroesophageal reflux, 4 with mild reflux on ultrasound (1 while coughing and another while crying) but not on fluoroscopy, and 1 with massive reflux and hiatal hernia. No side effects were reported.

Conclusions: Contrast-enhanced ultrasound allows confirming or ruling out the presence of GER and hiatal hernia. Its great advantage is the absence of ionizing radiation and the possibility of a real-time study, making it easier to detect reflux.

目的:探讨超声造影对胃食管反流及裂孔疝的诊断价值,避免电离辐射。材料与方法:对5个月~ 3岁的儿童进行7次食管胃超声造影检查,其中5次用于评估胃食管反流和裂孔疝,2次用于评估胃食管反流。我们使用了一种具有特定的对比度可视化软件的超声机,一种多频探头和超声造影剂。4例与上消化道相关。结果:7例患者中,4例正在等待经皮内镜胃造口术(2例合并鼻胃管,2例合并呕吐,其中1例合并鼻胃管),其余3例已行手术治疗:1例为幽门肥厚性狭窄,1例为食管闭锁,均合并呕吐;最后一例为经皮内镜胃造口术患者,疑似胃食管反流病。观察如下:2例无胃食管反流,4例超声检查有轻度反流(咳嗽时1例,哭诉时1例),透视检查无,1例有大量反流并裂孔疝。没有副作用的报道。结论:超声造影可以确认或排除GER和裂孔疝的存在。它的最大优点是没有电离辐射和实时研究的可能性,使其更容易检测反流。
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引用次数: 0
Implementation of a safety protocol for removal of bars in the pectus excavatum. 漏斗胸内铁条移除安全规程的实施。
J P Camacho, C Korzin, P A Lobos, G R Elmo

Introduction: Extraction of bars during Minimally Invasive Repair for Pectus Excavatum (MIRPE) is susceptible to complications ranging from mild to severe. Objective: to compare the outcomes following the implementation of a Bar Extraction Safety Protocol (BESP) to determine its effectiveness in reducing complications.

Material and methods: Retrospective comparative cohort study. Inclusion criteria: Patients who underwent bar removal from November 2013 to March 2024, in whom BESP was implemented, compared with a historical cohort operated on previously. Protocol includes: a) Preoperative measures: during implantation, use of smooth bars, a minimum of two bars with lateral stabilizers, sternal elevation; pre-removal: chest X-rays. b) Intraoperative measures: bilateral incisions, bar straightening, and "safety string" maneuver. c) Postoperative measures: 24-hour admission to the pediatric intensive care unit (PICU) and post-removal chest X-ray. Complications were classified using the Clavien-Dindo (C-D) system, focusing on clinically relevant ones (C-D ≥ II). Statistical analysis was performed using the chi-squared test (Stata v16).

Results: Sixty-seven patients were included; 62 were male. Mean age at surgery: 17 years (range 14-24). Thirty patients (43 bars) operated on pre-BESP, 37 (81 bars) post-BESP. Pre-BESP bars were serrated; post-BESP bars were smooth. Bar dwell time was 27 months (IQR 23-33) pre-BESP and 24 months (IQR 23-25) post-BESP. Hospital stay was 1 day in both groups (maximum: 16 vs. 4 days). Clinically relevant complications occurred in 20% of pre-BESP patients and 0% post-BESP (p=0.048).

Conclusions: Implementation of BESP significantly reduced complications after bar removal, improving safety during bar removal in the MIRPE.

导言:在微创修复漏斗胸(MIRPE)过程中拔棒容易产生从轻微到严重的并发症。目的:比较棒拔出安全方案(BESP)实施后的结果,以确定其在减少并发症方面的有效性。材料和方法:回顾性比较队列研究。纳入标准:2013年11月至2024年3月实施了BESP的患者,与先前手术的历史队列进行比较。方案包括:a)术前措施:植入期间,使用平滑棒,至少两根带侧稳定器的棒,胸骨抬高;术前:胸部x光片。b)术中措施:双侧切口、棒材矫直、“安全绳”操作。c)术后措施:儿童重症监护病房(PICU) 24小时住院,术后胸部x光检查。采用Clavien-Dindo (C-D)系统对并发症进行分类,重点关注临床相关的并发症(C-D≥II)。统计学分析采用卡方检验(Stata v16)。结果:纳入67例患者;男性62人。平均手术年龄:17岁(范围14-24岁)。30例(43条)患者行besp前手术,37例(81条)患者行besp后手术。Pre-BESP棒呈锯齿状;后besp棒是光滑的。实验前和实验后分别停留27个月(IQR 23-33)和24个月(IQR 23-25)。两组患者住院时间均为1天(最长16天vs. 4天)。临床相关并发症发生率为20%的besp前和0%的besp后(p=0.048)。结论:实施BESP可显著减少骨棒取出后的并发症,提高MIRPE骨棒取出过程中的安全性。
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引用次数: 0
Mediastinal teratoma as a cause of pleural effusion. 纵膈腔畸胎瘤是胸腔积液的原因。
F Imaz, P Garrido, M Medin, S Fiorini, A Santangelo, P Marino, R Godoy

Introduction: Teratomas are congenital tumors originating from pluripotent germ cells that contain derivatives of the three germ layers. One of their extragonadal forms is the anterior mediastinum, where they can present atypically.

Clinical case: A 3-year-old female patient was diagnosed with a cystic mediastinal teratoma in the context of pleural effusion due to tumor rupture. With negative tumor markers, surgical resolution was performed, which evidenced a heterogeneous tumor, adhered to adjacent structures, that was completely resected. The histopathology was compatible with a mature teratoma presenting pancreatic tissue, whose enzymatic secretion we assume was responsible for its rupture.

Conclusions: Cystic mediastinal teratomas can rupture and present as pleural effusion. Although this form of presentation is unusual, it is necessary to be aware of it for proper medical and surgical management.

畸胎瘤是一种先天性肿瘤,起源于多能生殖细胞,含有三种胚层的衍生物。其中一种角外形式是前纵隔,在那里它们可以表现得不典型。临床病例:一名3岁女性患者因肿瘤破裂并发胸腔积液,被诊断为囊性纵隔畸胎瘤。阴性肿瘤标志物,手术解决,证明异质肿瘤,粘附邻近结构,被完全切除。组织病理学与胰腺组织成熟畸胎瘤一致,我们认为其酶分泌是导致其破裂的原因。结论:囊性纵隔畸胎瘤可破裂并表现为胸腔积液。虽然这种形式的表现是不寻常的,有必要意识到它的适当的医疗和外科治疗。
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引用次数: 0
Surgical decision-making strategies in preterm neonates with necrotizing enterocolitis: A randomized controlled clinical trial. 新生儿坏死性小肠结肠炎的手术决策策略:一项随机对照临床试验。
G Fernández Ortega, M D Martín De Saro, S A Cuevas Covarrubias, G C Morón García, L Plaza Benhumea

Introduction: Surgical necrotizing enterocolitis (NEC) is associated with high mortality. Bell's criteria may indicate surgery in late stages, leading to worse outcomes. Early identification of surgical candidates is necessary. Objective: To evaluate surgical decision-making strategies in neonates with NEC, comparing Bell's criteria versus the combined use of metabolic disorder components (MD7) and paracentesis.

Material and methods: A randomized controlled clinical trial was conducted in perinatal hospitals (2022-2023), ClinicalTrials.gov identifier: NCT06035848. Preterm neonates with NEC were divided into a control group (CG): surgery determined by Bell's criteria, and an intervention group (IG): surgery indicated by MD7 and positive paracentesis. The primary outcome was mortality. Descriptive and inferential statistics, relative risk (RR), with 95% confidence interval (CI), and a p-value < 0.05 indicating statistical significance were used.

Results: 117 patients participated (CG n = 56, IG n = 61). In surgical NEC, mortality was 32.1% in IG and 64.7% in CG (p = 0.034; RR = 2, 95% CI: 1.1-4.8). In the IG, surgery was performed early, with better outcomes for perforation, intestinal necrosis, and reoperation (p < 0.05). There were no complications derived from paracentesis, and there were 3 non-therapeutic laparotomies in the IG.

Conclusions: The strategy based on MD7 and paracentesis was superior to Bell's criteria for guiding surgical decisions in preterm neonates with NEC, reducing mortality. The main limitation was a small number of non-therapeutic laparotomies, expected due to the greater sensitivity of the strategy.

手术坏死性小肠结肠炎(NEC)死亡率高。贝尔的标准可能表明在晚期进行手术,导致更糟糕的结果。早期识别手术候选人是必要的。目的:评价新生儿NEC的手术决策策略,比较Bell标准与联合使用代谢紊乱成分(MD7)和穿刺。材料和方法:在围产期医院进行随机对照临床试验(2022-2023),ClinicalTrials.gov标识符:NCT06035848。将NEC早产儿分为对照组(CG)和干预组(IG),对照组根据Bell标准进行手术,干预组根据MD7和穿刺阳性指示进行手术。主要结局是死亡率。描述性和推断性统计,相对危险度(RR), 95%可信区间(CI)和p值结果:117例患者参与(CG n = 56, IG n = 61)。在手术NEC中,IG的死亡率为32.1%,CG的死亡率为64.7% (p = 0.034; RR = 2, 95% CI: 1.1-4.8)。在IG中,手术早期进行,穿孔、肠坏死和再手术的预后较好(p)结论:基于MD7和穿刺的策略优于Bell的指导NEC早产儿手术决策的标准,降低了死亡率。主要的限制是少数非治疗性剖腹手术,由于该策略的敏感性更高。
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引用次数: 0
Conservative management of pediatric patients with solid organ injury after blunt abdominal trauma. Consensus sponsored by the Spanish Society of Pediatric Surgery. 小儿钝性腹部外伤后实体器官损伤的保守治疗。由西班牙儿科外科学会赞助的共识。
J Jiménez Gómez, R Rojo Díez, J C Moreno Alfonso, S Santiago Martínez, C Ruiz Hierro, C Barceló Cañellas, C Leganés Villanueva, G Roberto Lorenzo, A Laín Fernández, R Gander, J Lluna González, J C de Agustín Asensio, A Prieto Campo, M Prada Arias, J R Gómez-Veiras

Introduction: The treatment of choice in pediatrics for solid organ injuries resulting from blunt abdominal trauma is conservative management. However, in Spain, said management has proven to be heterogeneous and inconsistent with recent evidence. The Spanish Society of Pediatric Surgery (SECP) sponsored the development of this consensus document in the year 2024.

Material and methods: After recruiting a group of experts, a bibliographic review was conducted using the systematic reviews from APSA, the ATOMAC group, and contributions from the experts themselves, to draft a series of initial suggestions. The experts, using the Delphi method, scored these (Likert scale) in different voting rounds until statistical stability in the responses was determined (Wilcoxon Test). Consensus was defined as agreement (scores 4-5) exceeding 70%.

Results: Twelve experts from 11 centers were recruited, achieving gender parity. Seventeen suggestions were developed, organized into four key areas: PICU admission, discharge criteria and strict bed rest, analytical/radiological controls, and physical activity restriction. Statistical stability was reached after 2 scoring rounds, achieving consensus on 15 of the 17 suggestions, while it was not achieved regarding hospital discharge criteria and duration of strict bed rest.

Conclusions: Through Delphi methodology, a consensus document on the conservative management of solid organ injuries was developed. Although consensus was not reached on key aspects such as discharge criteria and strict bed rest, this document aims to help homogenize clinical practice.

儿科钝性腹部外伤所致实体器官损伤的治疗选择是保守治疗。然而,在西班牙,管理层已被证明是异质的,与最近的证据不一致。西班牙儿科外科学会(SECP)在2024年赞助了这一共识文件的制定。材料和方法:在招募了一批专家后,利用来自APSA、ATOMAC小组的系统评论和专家们自己的贡献进行了文献审查,起草了一系列初步建议。专家们使用德尔菲法,在不同的投票轮中对这些(李克特量表)进行评分,直到确定了回答的统计稳定性(Wilcoxon Test)。共识定义为同意(得分4-5)超过70%。结果:从11个中心招募了12名专家,实现了性别平等。提出了17项建议,分为四个关键领域:PICU入院、出院标准和严格卧床休息、分析/放射控制和身体活动限制。2轮评分后达到统计稳定,17项建议中有15项意见达成一致,出院标准和严格卧床时间未达成一致。结论:通过德尔菲法,对实体器官损伤的保守治疗达成共识。虽然在出院标准和严格卧床休息等关键方面尚未达成共识,但本文件旨在帮助统一临床实践。
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引用次数: 0
Median arcuate ligament syndrome: Successful laparoscopic management enhanced by indocyanine green fluorescence. 正中弓状韧带综合征:吲哚菁绿荧光增强腹腔镜成功治疗。
F Ortiz, M Cadario, M Carreño, C M Pérez Espinosa, J Ruiz, J Kaplan, C Martín, V H Ayarzabal

Introduction: Median arcuate ligament syndrome (MALS) is a rare vascular disorder characterized by compression of the celiac artery by the median arcuate ligament, resulting in variable clinical presentations, including abdominal pain.

Case report: A 16-year-old female patient presented with chronic abdominal pain, recurrent vomiting, and weight loss over seven months. Doppler ultrasound showed increased peak systolic velocity in the celiac artery (331 cm/s) exacerbated during expiration and computed tomography angiography confirmed celiac artery compression with the typical "J-hook" deformity. She underwent successful laparoscopic surgical decompression assisted with indocyanine green fluorescence to confirm adequate perfusion of the celiac territory. Pre and postoperative quality of life was assessed using PedsQL scores, showing significant improvement. After a six-month follow-up, the patient remains asymptomatic.

Conclusion: This case highlights the importance of considering MALS in the differential diagnosis of chronic abdominal pain in adolescents and reinforces the established efficacy and safety of laparoscopic management for MALS.

简介:正中弓韧带综合征(MALS)是一种罕见的血管疾病,其特征是正中弓韧带压迫腹腔动脉,导致多种临床表现,包括腹痛。病例报告:一名16岁的女性患者表现为慢性腹痛,反复呕吐,体重下降超过7个月。多普勒超声显示腹腔动脉收缩速度峰值增加(331 cm/s),呼气时加剧,计算机断层血管造影证实腹腔动脉受压,呈典型的“j钩”形畸形。她在吲哚菁绿荧光辅助下成功行腹腔镜手术减压,以确认腹腔区域灌注充足。使用PedsQL评分评估术前和术后生活质量,显示有显著改善。六个月的随访后,患者仍无症状。结论:本病例强调了青少年慢性腹痛鉴别诊断中考虑肌萎缩侧索硬化症的重要性,并加强了腹腔镜治疗肌萎缩侧索硬化症的有效性和安全性。
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引用次数: 0
PedIAtric Surgery: Artificial Intelligence and the new horizon in Science. 儿科外科:人工智能和科学的新视野。
B Núñez García
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引用次数: 0
Ankyloglossia and frenotomy in Spain: assessment by the pediatric surgeon. 西班牙的强直性咬合和截骨术:由儿科外科医生评估。
I Carrillo Arroyo, C Rico Espiñeira, M Rico Jiménez, H Souto Romero, J L Alonso Calderón
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引用次数: 0
Low-cost simulation model for the treatment of inguinal hernia in pediatric patients. 小儿腹股沟疝治疗的低成本模拟模型。
R Ramírez Díaz, A Sáenz Dorado, C Martínez Paz, L S Gallego, R I Cavaco Fernandes, J Bueno Recio

Introduction: Open inguinal herniorrhaphy is the most widely used inguinal hernia repair treatment in pediatric surgery. Understanding the anatomy of the inguinal canal is essential to conduct this procedure.

Material and methods: A low-cost, easily reproducible model aimed at training residents was designed to simulate open inguinal herniorrhaphy in children. It simulated the presence of inguinal hernia using low-cost materials such as balloons, transparent dressings, materials simulating the cremaster muscle, and cord elements. The model was validated by 4 pediatric surgeons and built by residents, who completed a post-simulation survey using the Likert scale. The survey assessed general aspects such as usefulness, texture, or grade of recommendation for regular training.

Results: The surgical technique was completed step-by-step in the simulation model by a total of 10 pediatric surgery residents from various Spanish hospitals. Scores of 4.5/5 in general aspects, 4/5 in texture, 4.7/5 in usefulness, and 4.6/5 in grade of recommendation were achieved.

Conclusions: We believe this low-cost, easy-to-build model stands as a useful tool both for surgical training and for understanding the anatomy of the inguinal canal in training residents.

腹股沟开放性疝修补术是小儿外科中应用最广泛的一种腹股沟疝修补术。了解腹股沟管的解剖结构是进行该手术的必要条件。材料和方法:设计了一个低成本、易于复制的模型,用于培训住院医师,以模拟儿童开放式腹股沟疝修补术。它使用低成本的材料,如气球、透明敷料、模拟肌群的材料和脐带元素来模拟腹股沟疝的存在。该模型由4名儿科外科医生验证,并由住院医师建立,他们使用李克特量表完成了模拟后的调查。该调查评估了一般方面,如有用性、质地或推荐定期培训的等级。结果:在模拟模型中,共有10名来自西班牙各医院的儿科外科住院医师逐步完成了手术技术。总体评分为4.5/5,质地评分为4/5,实用性评分为4.7/5,推荐等级评分为4.6/5。结论:我们相信这种低成本、易于构建的模型对于外科培训和住院医师了解腹股沟管的解剖结构都是一种有用的工具。
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引用次数: 0
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Cirugia pediatrica : organo oficial de la Sociedad Espanola de Cirugia Pediatrica
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