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Neonatal Riga-Fede disease: A systematic review of 84 cases collected over 120 years 新生儿里加-费德病:对 120 年来收集的 84 个病例的系统回顾
Q4 Medicine Pub Date : 2024-07-28 DOI: 10.47338/jns.v13.1293
Rangapriya Alagar Thirumalai, V. Raveenthiran
Background: Traumatic ulceration of the tongue due to congenital teeth is known as Riga-Fede disease (RFD). The literature on RFD consists largely of anecdotal case reports, thereby lacking valid clinical recommendations. While managing a newborn with RFD complicated by Ludwig’s angina, this knowledge gap was recognized. Therefore, this systematic review was conducted to gain insight into the optimal management and the possible complications of RFD.Methods: A literature search was conducted using PubMed, Google Scholar, and Science Direct, supplemented with African Journals Online, Indmed, and manual searches. Papers published between 1900 and July 2023 in any language were retrieved. Neonates with natal or neonatal teeth and ulceration of the oral mucosa were included. Papers with incomplete data and review articles were excluded.Results: A total of 84 newborns with RFD from 62 published reports (including the case reported herein) were analyzed. Most of the reports were from the Indian subcontinent, and the frequency of reporting has increased since the year 2000. Tongue ulceration in all cases was due to mandibular incisors. There was no significant difference in the median duration of healing between the tooth extraction group (14 days) and the conservative management group (18 days). There was only a very weak positive correlation between the duration of symptoms and healing time, which was statistically insignificant.Conclusion: Healing of mucosal ulcers in RFD is often protracted and does not appear to be influenced by the duration of symptoms or by the modality of treatment. Extraction of the offending teeth is more common (75% of cases) than conservative management, such as grinding (ameloplasty) or resin capping of teeth. RFD is mostly a benign disorder; however, serious complications such as meningitis, Ludwig’s angina, and soft tissue abscesses can rarely occur with Staphylococcus aureus infections.
背景:先天性牙齿导致的舌外伤性溃疡被称为里加-费德病(Riga-Fede disease,RFD)。有关 RFD 的文献主要由轶事病例报告组成,因此缺乏有效的临床建议。在治疗一名患有 RFD 并发路德维希心绞痛的新生儿时,我们认识到了这一知识空白。因此,我们进行了此次系统性综述,以深入了解 RFD 的最佳治疗方法和可能出现的并发症:方法:使用 PubMed、Google Scholar 和 Science Direct 进行文献检索,并辅以非洲期刊在线、Indmed 和人工检索。检索了 1900 年至 2023 年 7 月间发表的任何语言的论文。有新生儿牙齿或新生儿口腔粘膜溃疡的新生儿也包括在内。数据不完整的论文和综述性文章被排除在外:结果:共分析了 62 篇已发表报告中的 84 例 RFD 新生儿(包括本文报告的病例)。大多数报告来自印度次大陆,自 2000 年以来,报告的频率有所增加。所有病例的舌头溃疡都是由于下颌切牙引起的。拔牙组(14 天)和保守治疗组(18 天)的中位愈合时间没有明显差异。症状持续时间与愈合时间之间只有很弱的正相关,在统计学上不显著:结论:RFD 患者黏膜溃疡的愈合时间通常较长,似乎不受症状持续时间或治疗方式的影响。与磨牙(臼齿成形术)或树脂盖牙等保守治疗方法相比,拔除患牙(75% 的病例)更为常见。RFD 多为良性疾病,但金黄色葡萄球菌感染很少会出现脑膜炎、路德维希心绞痛和软组织脓肿等严重并发症。
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引用次数: 0
Are subclavian percutaneous central venous catheters an alternative to Hickman-Broviac catheters in neonates and young infants? 在新生儿和婴幼儿中,锁骨下经皮中心静脉导管是否可替代希克曼-布罗维克导管?
Q4 Medicine Pub Date : 2024-07-20 DOI: 10.47338/jns.v13.1304
Anouar Jarraya, Manel Kammoun, Olfa Cherif, Mariem Ben Abdallah
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引用次数: 0
Situs ambiguous with duodenal atresia in a neonate: A case report with literature review 一名患有十二指肠闭锁的新生儿病位不清:病例报告与文献综述
Q4 Medicine Pub Date : 2024-07-20 DOI: 10.47338/jns.v13.1318
O. Edan
Background: Situs ambiguous is a rare congenital anomaly characterized by an unusual and irregular distribution of the major visceral organs and vessels within the chest and abdomen. In this condition, the organs are arranged in a way that cannot be classified as either situs inversus or situs solitus. We report this case due to the rarity of this anomaly and its uncommon association with duodenal atresia in a neonate.Case Presentation: A full-term newborn female presented with repeated bilious vomiting and failure to pass meconium for five days. An erect abdominal X-ray revealed a double-bubble sign in the left hemiabdomen, and a CT scan confirmed an abnormal relationship between the liver and stomach. Operative exploration confirmed the diagnosis of situs ambiguous with duodenal atresia, which was treated with a diamond duodenoduodenostomy.Conclusion: Situs ambiguous is a rare variant of situs anomaly with few reported cases worldwide. It has two subtypes: left and right isomerism, based on the anatomy of the spleen. Clinically, it can present with features of intestinal obstruction when associated with duodenal atresia.
背景:坐位不清是一种罕见的先天性畸形,其特点是主要内脏器官和血管在胸部和腹部的分布异常且不规则。在这种情况下,器官的排列方式无法被归类为坐位不清或坐位固有。我们之所以报告这个病例,是因为这种畸形非常罕见,而且与新生儿十二指肠闭锁有关:一名足月新生女婴因反复胆汁性呕吐和连续五天未排出胎粪而就诊。直立腹部 X 光片显示左半腹有双气泡征,CT 扫描证实肝脏和胃之间的关系异常。手术探查确诊为坐位不清伴十二指肠闭锁,采用菱形十二指肠造口术进行治疗:结论:坐位不清症是一种罕见的坐位异常变异,全球鲜有报道。它有两个亚型:根据脾脏的解剖结构分为左侧和右侧异位。在临床上,如果伴有十二指肠闭锁,则可能出现肠梗阻的特征。
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引用次数: 0
Inguinal hernia repair in newborns: A systematic literature review 新生儿腹股沟疝修补术:系统性文献综述
Q4 Medicine Pub Date : 2024-05-20 DOI: 10.47338/jns.v13.1253
R. Lamiri, Fatma Chebab, N. Kechiche, S. Mani, Hayet Ben Hmida, Maroua El Ouaer, Sawsen Chakroun, A. Ksia, M. Mekki, M. Belghith, Nahla Hmidi, L. Sahnoun
Background: Inguinal hernia (IH) is a prevalent condition in children necessitating surgical repair. However, determining the optimal timing (early or delayed) of inguinal herniotomy in neonates and preterm infants remains debatable. While open herniotomy traditionally serves as the standard inguinal hernia repair (IHR) procedure, laparoscopic repair has gained traction in recent decades. Our study aims to scrutinize both the optimal timing and approach to inguinal hernia repair in neonates and preterm infants.Methods: We conducted a literature review on surgical repair of IH in neonates and preterm infants published between 1999 and 2024.Results: Twenty studies met the inclusion criteria for this review. All studies were retrospective, predominantly originating from Europe and the United States. Thirteen out of the 20 studies focused solely on preterm infants. Patient sample sizes ranged from 30 to 8037, totaling 14533 patients. Most studies indicate that delaying inguinal hernia repair in newborns and preterm infants does not increase the risk of incarceration or recurrence. Moreover, postponing repair until after discharge from the NICU correlates with a shorter postoperative hospital stay and notably diminishes the risk of long-term postoperative ventilator dependence, thereby mitigating potential perioperative complications. Hence, this approach seems safe for certain patients whose families can reliably access appropriate surgical care.Conclusion: Significant disparities exist in the timing of inguinal hernia repair for newborns and preterm infants across various pediatric surgery centers. Current evidence suggests delayed inguinal hernia repair may be a viable option for selected patients. Regarding the optimal approach in this population, laparoscopy appears safe and effective.
背景:腹股沟疝气(IH)是一种需要手术修补的儿童常见疾病。然而,确定新生儿和早产儿腹股沟疝气切除术的最佳时机(早期或延迟)仍存在争议。传统上,开腹疝气切除术是腹股沟疝气修补术(IHR)的标准术式,而近几十年来,腹腔镜修补术越来越受到重视。我们的研究旨在探讨新生儿和早产儿腹股沟疝修补术的最佳时机和方法:我们对 1999 年至 2024 年间发表的有关新生儿和早产儿腹股沟疝手术修复的文献进行了回顾:结果:20 项研究符合本综述的纳入标准。所有研究均为回顾性研究,主要来自欧洲和美国。20 项研究中有 13 项仅针对早产儿。患者样本量从 30 到 8037 不等,共计 14533 名患者。大多数研究表明,推迟新生儿和早产儿腹股沟疝修补术并不会增加嵌顿或复发的风险。此外,将修复手术推迟到新生儿重症监护室出院后进行,可缩短术后住院时间,并显著降低术后长期依赖呼吸机的风险,从而减轻围手术期的潜在并发症。因此,这种方法对于某些患者来说似乎是安全的,因为他们的家人可以可靠地获得适当的手术护理:结论:各儿科手术中心在新生儿和早产儿腹股沟疝修补术的时机选择上存在显著差异。目前的证据表明,延迟腹股沟疝修补术可能是部分患者的可行选择。关于这一人群的最佳方法,腹腔镜手术似乎安全有效。
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引用次数: 0
Apple-Peel intestinal atresia: A systematic review 苹果脆皮肠闭锁:系统综述
Q4 Medicine Pub Date : 2024-05-14 DOI: 10.47338/jns.v13.1255
Francesco Laconi, Charline Bischoff, Theo Michailos, Ongoly Okiemy M'Inserret, Emmanuelle Kremer, Marie-Laurence Poli-Merol
Background: Apple-peel bowel atresia (APA) is rare and thus lacks proper management guidelines. This systematic review analyzes various interventions found in the literature for this type of atresia and highlights the outcomes of each intervention with their complications.Methods: The relevant literature on PubMed, Scopus, Embase, and Cochrane was reviewed from November 2020 to January 2021. Articles published in English, French, Italian, or Spanish between 1990 and 2020, focusing on APA, were included. Data on demography, clinical profile, management provided, time to achieve full feed, length of hospital stay, complications, etc., reviewed.Results: A total of 2495 articles were found, of which only 48 met the inclusion criteria. Among these, 125 patients were treated, with 15 deaths. The most frequently employed intervention was primary resection, with or without tapering, while ostomy creation was less commonly used. The most frequent complication across all techniques appeared to be cholestasis, followed by stenosis of the anastomosis and sepsis. Additionally, there were a total of 7 cases of short bowel syndrome (SBS).Conclusion: The management of apple-peel atresia seems variable in various centers of different countries. The review failed to assess which interventions could lead to faster full enteral feeding. The distal part of the anastomosis must be considered as an active part in the resumption of bowel function.
背景:苹果皮肠闭锁(APA)非常罕见,因此缺乏适当的管理指南。这篇系统性综述分析了文献中发现的针对这种类型闭锁的各种干预措施,并强调了每种干预措施的结果及其并发症:方法:回顾了 2020 年 11 月至 2021 年 1 月期间在 PubMed、Scopus、Embase 和 Cochrane 上的相关文献。纳入了 1990 年至 2020 年间以英文、法文、意大利文或西班牙文发表的以 APA 为主题的文章。审查了人口统计学、临床概况、提供的管理、实现完全进食的时间、住院时间、并发症等方面的数据:结果:共找到 2495 篇文章,其中只有 48 篇符合纳入标准。其中,125 名患者接受了治疗,15 人死亡。最常用的干预方法是原发性切除术,包括或不包括锥切术,而造口术则较少使用。所有技术中最常见的并发症似乎是胆汁淤积,其次是吻合口狭窄和败血症。此外,共有 7 例出现短肠综合征(SBS):结论:在不同国家的不同中心,对苹果皮闭锁的处理似乎各不相同。综述未能评估哪些干预措施可加快完全肠内喂养的速度。吻合口的远端必须被视为恢复肠道功能的一个积极部分。
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引用次数: 0
A remarkable case of intrauterine intussusception, ileal atresia, and complicated meconium ileus: A case report 一例罕见的宫内肠套叠、回肠闭锁和复杂胎粪回流病例:病例报告
Q4 Medicine Pub Date : 2024-05-07 DOI: 10.47338/jns.v13.1243
Jonathan Hencke, O. Diez, Steffan Loff
Background: Intrauterine intussusception, although very rare (<2%), is a potential cause of intestinal atresia. It usually manifests as bowel obstruction during the first hours or days of life.Case Presentation: We report a case with fetal ascites prompting early Cesarean section, with subsequent percutaneous abdominal drainage and laparotomy. Intraoperative findings showed meconium peritonitis and type IIIa ileal atresia with intussusception of the distal part, with the necrosed intussusceptum likely causing perforation and ileal atresia; in addition, the distal ileum demonstrated signs of meconium ileus. After ileostomy creation, the postoperative course was uneventful. Cystic fibrosis was excluded via a sweat test.Conclusion: Prompt diagnosis and management of neonatal intestinal obstruction secondary to the unusual combination of fetal ascites, intussusception, complicated meconium ileus, and ileal atresia results in a favorable outcome.
背景:宫内肠套叠虽然非常罕见(<2%),但却是导致肠闭锁的潜在原因之一。它通常在婴儿出生后数小时或数天内表现为肠梗阻:我们报告了一例因胎儿腹水而导致早期剖宫产的病例,随后进行了经皮腹腔引流和开腹手术。术中发现胎粪腹膜炎和 IIIa 型回肠闭锁,远端有肠套叠,坏死的肠套叠可能导致穿孔和回肠闭锁;此外,远端回肠有胎粪回流的迹象。在建立回肠造口术后,术后恢复顺利。通过出汗试验排除了囊性纤维化:结论:新生儿肠梗阻继发于胎儿腹水、肠套叠、复杂性胎粪回肠症和回肠闭锁的不寻常组合,及时诊断和处理可获得良好的预后。
{"title":"A remarkable case of intrauterine intussusception, ileal atresia, and complicated meconium ileus: A case report","authors":"Jonathan Hencke, O. Diez, Steffan Loff","doi":"10.47338/jns.v13.1243","DOIUrl":"https://doi.org/10.47338/jns.v13.1243","url":null,"abstract":"Background: Intrauterine intussusception, although very rare (<2%), is a potential cause of intestinal atresia. It usually manifests as bowel obstruction during the first hours or days of life.\u0000Case Presentation: We report a case with fetal ascites prompting early Cesarean section, with subsequent percutaneous abdominal drainage and laparotomy. Intraoperative findings showed meconium peritonitis and type IIIa ileal atresia with intussusception of the distal part, with the necrosed intussusceptum likely causing perforation and ileal atresia; in addition, the distal ileum demonstrated signs of meconium ileus. After ileostomy creation, the postoperative course was uneventful. Cystic fibrosis was excluded via a sweat test.\u0000Conclusion: Prompt diagnosis and management of neonatal intestinal obstruction secondary to the unusual combination of fetal ascites, intussusception, complicated meconium ileus, and ileal atresia results in a favorable outcome.","PeriodicalId":34201,"journal":{"name":"Journal of Neonatal Surgery","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-05-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141003570","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
Attrition rate among patients of Anorectal Malformations on colostomy: A matter of concern 接受结肠造口术的肛门直肠畸形患者的流失率:一个值得关注的问题
Q4 Medicine Pub Date : 2024-05-06 DOI: 10.47338/jns.v13.1306
Parveen Kumar, Sauradeep Dey, Ashvin Damdoo, Shishir Kumar, V. Manchanda
Background: Anorectal malformations (ARM) are correctable congenital malformations with good prognosis. Traditionally it is managed by staged procedure, with diversion colostomy, followed by definitive procedure and stoma reversal in the third stage. It is recommended to complete all stages of repair by 6 months of age for better long-term continence. We had fewer patients coming for definitive surgery and stoma reversal than the patients undergoing colostomy for ARM, so we planned this study to find out the attrition rate among patients undergoing stoma for ARM and explore the reasons for the same.Methods: An observational study was conducted in the Pediatric Surgery department of Chacha Nehru Bal Chikitsalaya, New Delhi. All the case records of patients with ARM who underwent diversion stoma at our center from January 2018 to December 2019 were retrieved. Further follow-up case records were retrieved with the same Unique Health Identification Number (UHID). When the definitive surgery/ stoma closure was not found in the hospital records till December 2022, the parents were contacted telephonically to find out if the children had undergone definitive surgery at some other center, were awaiting surgery, or had died. The attrition was calculated as the difference between the number of patients for whom a stoma was done and the number of patients who underwent definitive surgery and stoma closure till the data acquisition.Results: A total of 105 patients were included in the study with male preponderance. Colostomy was done for 73.33% of patients in the neonatal period (77/105). The cloacal malformation was found in 7 (6.67%), rectal atresia in 6 (5.71%), and 9 had congenital pouch colon (8.57%). The age at stoma creation for ARM ranged from 1 day to 9.3 years (mean 0.67 ±1.96 years). Cardiac and renal anomalies were among the major associations. 28 of the neonates (26.67%) had stomal complications. Stomal issues were reported in 32.4%. Mortality was reported in 37 patients (35.24%) including 35 neonates. The attrition rate in our study was found to be 38.24% (26 out of 68). Factors contributing to attrition range from anemia and protracted waiting lists to the anesthesiologist’s pursuit of optimal patient stabilization, alongside familial or personal matters faced by caregivers.Conclusion: There was a high attrition rate in operated patients of ARM in whom stoma was constructed. The main reasons for the same were anemia and a long surgical waiting list.
背景:肛门直肠畸形(ARM)是一种可矫正的先天性畸形,预后良好。传统的处理方法是分期手术,先进行分流结肠造口术,然后在第三期进行最终手术和造口翻转术。我们建议患者在 6 个月大之前完成所有阶段的修复手术,以便更好地保持长期排便通畅。与因 ARM 而接受结肠造口术的患者相比,前来接受最终手术和造口翻转术的患者较少,因此我们计划开展这项研究,以了解因 ARM 而接受造口术的患者的流失率,并探讨其原因:方法:我们在新德里 Chacha Nehru Bal Chikitsalaya 小儿外科开展了一项观察性研究。检索了 2018 年 1 月至 2019 年 12 月期间在本中心接受分流造口术的所有 ARM 患者的病例记录。通过相同的唯一健康识别码(UHID)检索了进一步的随访病例记录。如果到 2022 年 12 月仍未在医院记录中找到明确手术/造口关闭的病例,则电话联系患儿家长,以了解患儿是否在其他中心接受了明确手术、正在等待手术或已经死亡。自然减员的计算方法是:在数据采集前,已完成造口手术的患者人数与已完成手术并关闭造口的患者人数之差:共有 105 名患者参与研究,其中男性居多。73.33%的患者在新生儿期进行了结肠造口术(77/105)。发现泄殖腔畸形的有 7 例(6.67%),直肠闭锁的有 6 例(5.71%),先天性袋状结肠的有 9 例(8.57%)。ARM 造口的年龄从 1 天到 9.3 岁不等(平均 0.67 ± 1.96 岁)。心脏和肾脏异常是主要的关联因素。28名新生儿(26.67%)有造口并发症。32.4%的新生儿有气孔问题。37名患者(35.24%)死亡,其中包括35名新生儿。我们的研究发现流失率为 38.24%(68 例中有 26 例)。导致减员的因素包括贫血、等待时间过长、麻醉师追求患者最佳稳定状态,以及护理人员面临的家庭或个人问题:结论:在造口手术的 ARM 患者中,自然减员率很高。结论:造口手术的 ARM 患者流失率很高,主要原因是贫血和手术等待时间过长。
{"title":"Attrition rate among patients of Anorectal Malformations on colostomy: A matter of concern","authors":"Parveen Kumar, Sauradeep Dey, Ashvin Damdoo, Shishir Kumar, V. Manchanda","doi":"10.47338/jns.v13.1306","DOIUrl":"https://doi.org/10.47338/jns.v13.1306","url":null,"abstract":"Background: Anorectal malformations (ARM) are correctable congenital malformations with good prognosis. Traditionally it is managed by staged procedure, with diversion colostomy, followed by definitive procedure and stoma reversal in the third stage. It is recommended to complete all stages of repair by 6 months of age for better long-term continence. We had fewer patients coming for definitive surgery and stoma reversal than the patients undergoing colostomy for ARM, so we planned this study to find out the attrition rate among patients undergoing stoma for ARM and explore the reasons for the same.\u0000Methods: An observational study was conducted in the Pediatric Surgery department of Chacha Nehru Bal Chikitsalaya, New Delhi. All the case records of patients with ARM who underwent diversion stoma at our center from January 2018 to December 2019 were retrieved. Further follow-up case records were retrieved with the same Unique Health Identification Number (UHID). When the definitive surgery/ stoma closure was not found in the hospital records till December 2022, the parents were contacted telephonically to find out if the children had undergone definitive surgery at some other center, were awaiting surgery, or had died. The attrition was calculated as the difference between the number of patients for whom a stoma was done and the number of patients who underwent definitive surgery and stoma closure till the data acquisition.\u0000Results: A total of 105 patients were included in the study with male preponderance. Colostomy was done for 73.33% of patients in the neonatal period (77/105). The cloacal malformation was found in 7 (6.67%), rectal atresia in 6 (5.71%), and 9 had congenital pouch colon (8.57%). The age at stoma creation for ARM ranged from 1 day to 9.3 years (mean 0.67 ±1.96 years). Cardiac and renal anomalies were among the major associations. 28 of the neonates (26.67%) had stomal complications. Stomal issues were reported in 32.4%. Mortality was reported in 37 patients (35.24%) including 35 neonates. The attrition rate in our study was found to be 38.24% (26 out of 68). Factors contributing to attrition range from anemia and protracted waiting lists to the anesthesiologist’s pursuit of optimal patient stabilization, alongside familial or personal matters faced by caregivers.\u0000Conclusion: There was a high attrition rate in operated patients of ARM in whom stoma was constructed. The main reasons for the same were anemia and a long surgical waiting list.","PeriodicalId":34201,"journal":{"name":"Journal of Neonatal Surgery","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141006014","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
Single-center experience of fetus in fetu: A case series 单中心胎儿治疗经验:病例系列
Q4 Medicine Pub Date : 2024-04-13 DOI: 10.47338/jns.v13.1279
Habachi Ghada, Sabolić Ivana, Mesic Marko, A. Ksia, Myriam Ben Fredj, S. Youssef, Sami Sfar, N. Kechiche, R. Lamiri, M. Mekki, M. Belghith, Marwa Messoud, S. Mosbahi, L. Sahnoun
Background: Fetus in fetu is a rare congenital malformation resulting from abnormal embryogenesis in a monochorionic diamniotic twinning gestation. This study aimed to document our experience with this anomaly in a developing country.Methods: This retrospective analysis covers cases of fetus in fetu diagnosed at our institution between 1999 and 2023. Patients presenting with an intracorporeal mass containing a vertebral column and an appropriate arrangement of organs and limbs around the axis were included in the study.Results: Five female patients with fetus in fetu were identified. The timing of diagnosis varied from incidental antenatal ultrasound discovery to postnatal abdominal mass presentation. The initial diagnosis involved radiological and serological investigations, confirmed by histopathology after surgical excision. The mean age at the time of surgery was 44 days. All patients underwent complete excision of the fetal mass, resulting in excellent postoperative outcomes and no recurrences.Conclusion: Fetus in fetu is a pediatric rarity and must be distinguished from a teratoma mass, which carries a high malignant potential. Histological and genetic studies may contribute to understanding the pathogenesis and etiology of the disease.
背景:胎儿畸形是一种罕见的先天性畸形,是单绒毛膜双胎妊娠中胚胎发育异常所致。本研究旨在记录我们在发展中国家处理这种畸形的经验:这项回顾性分析涵盖了1999年至2023年期间在我院确诊的胎儿畸形病例。研究对象包括出现包含椎体的体腔内肿块以及围绕轴线适当排列的器官和肢体的患者:结果:共发现五名女性胎儿患者。诊断时间从产前超声偶然发现到产后出现腹部肿块不等。初步诊断包括放射学和血清学检查,手术切除后经组织病理学确诊。手术时的平均年龄为 44 天。所有患者均完全切除了胎儿肿块,术后效果良好,无复发:结论:胎儿畸形是儿科罕见病,必须与畸胎瘤区分开来,后者具有很高的恶性潜能。组织学和遗传学研究可能有助于了解该病的发病机制和病因。
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引用次数: 0
Extremely low birth weight (ELBW) neonates for emergency surgery- A challenge for the Anesthesiologist: A case series 接受紧急手术的极低出生体重(ELBW)新生儿--麻醉医师面临的挑战:病例系列
Q4 Medicine Pub Date : 2024-03-14 DOI: 10.47338/jns.v13.1294
P. Varshney, Madhurima Sinharay, Anshu Gupta, M. Pandey
Background: Extremely Low Birth Weight (ELBW) newborns, defined as those with a birth weight of 1000 grams or less, present challenging anesthetic scenarios. Emergency surgeries in these infants elevate their vulnerability to various complications.Case Presentation: We present a case series involving 5 ELBW neonates who underwent emergency surgery. Three of them were at a post-conceptual age (PCA) of ≥ 33 weeks and underwent procedures for congenital birth defects, including esophageal atresia, ileal atresia, and gastroschisis. The remaining two neonates, with a PCA of 29-30 weeks, underwent surgery for necrotizing enterocolitis (NEC).Conclusion: The compromised physiology resulting from distinct disease processes and the underdeveloped systems of ELBW preterm neonates necessitate focused care and strategic anesthesia. This approach is crucial to mitigate morbidity and mortality risks in such vulnerable patients.
背景:极低出生体重儿(ELBW)是指出生体重在 1000 克或以下的新生儿,他们的麻醉情况极具挑战性。对这些婴儿进行紧急手术会使他们更容易出现各种并发症:我们展示了一个涉及 5 例接受紧急手术的 ELBW 新生儿的系列病例。其中 3 名新生儿的受孕年龄(PCA)≥ 33 周,接受了先天性出生缺陷手术,包括食道闭锁、回肠闭锁和胃裂。其余两名新生儿的 PCA 为 29-30 周,因坏死性小肠结肠炎(NEC)接受了手术:结论:ELBW 早产新生儿因不同的疾病过程和发育不全的系统而导致生理机能受损,因此有必要进行重点护理和策略性麻醉。这种方法对于降低此类脆弱患者的发病率和死亡率风险至关重要。
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引用次数: 0
Jarcho Levin Syndrome with open neural tube defect: A case report 贾乔-列文综合征伴开放性神经管缺陷:病例报告
Q4 Medicine Pub Date : 2024-02-26 DOI: 10.47338/jns.v13.1282
Md Mokarram Ali, Y. Kadian, Saima Ejaz, A. Kadian
Background: Jarcho Levin syndrome is a rare genetic disorder characterized by spine abnormalities and the absence of ribs. It is associated with various congenital malformations and carries significant morbidity and mortality. The simultaneous presentation of this syndrome with neural tube defects is seldom encountered.Case Presentation: We report a case of a 2-day-old male who presented with a lesion on his back since birth. Clinical and radiological examinations revealed thoracolumbar meningomyelocele with the absence of upper ribs on the right side and a patent foramen ovale. Subsequently, the patient underwent surgery for meningomyelocele, while the rib anomalies were managed conservatively.Conclusion: Jarcho Levin syndrome is a rare disorder, and its association with meningomyelocele is extremely uncommon. It should be considered in the evaluation of a patient with a neural tube defect and respiratory distress.
背景介绍贾乔-莱文综合征是一种罕见的遗传性疾病,以脊柱畸形和无肋骨为特征。它伴有各种先天性畸形,发病率和死亡率都很高。同时伴有神经管缺陷的病例很少见:我们报告了一例出生仅 2 天的男性病例。临床和放射学检查显示,患者胸腰椎脑膜疝,右侧上肋骨缺失,卵圆孔通畅。随后,患者接受了脑膜疝手术,而肋骨异常则采取了保守治疗:结论:贾尔乔-莱文综合征是一种罕见疾病,与脑膜瘤相关的病例极为罕见。结论:贾尔乔-莱文综合征是一种罕见疾病,与脑膜瘤相关的病例极为罕见,在评估有神经管缺陷和呼吸窘迫的患者时应考虑到这种疾病。
{"title":"Jarcho Levin Syndrome with open neural tube defect: A case report","authors":"Md Mokarram Ali, Y. Kadian, Saima Ejaz, A. Kadian","doi":"10.47338/jns.v13.1282","DOIUrl":"https://doi.org/10.47338/jns.v13.1282","url":null,"abstract":"Background: Jarcho Levin syndrome is a rare genetic disorder characterized by spine abnormalities and the absence of ribs. It is associated with various congenital malformations and carries significant morbidity and mortality. The simultaneous presentation of this syndrome with neural tube defects is seldom encountered.\u0000Case Presentation: We report a case of a 2-day-old male who presented with a lesion on his back since birth. Clinical and radiological examinations revealed thoracolumbar meningomyelocele with the absence of upper ribs on the right side and a patent foramen ovale. Subsequently, the patient underwent surgery for meningomyelocele, while the rib anomalies were managed conservatively.\u0000Conclusion: Jarcho Levin syndrome is a rare disorder, and its association with meningomyelocele is extremely uncommon. It should be considered in the evaluation of a patient with a neural tube defect and respiratory distress.","PeriodicalId":34201,"journal":{"name":"Journal of Neonatal Surgery","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-02-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140431356","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
期刊
Journal of Neonatal Surgery
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