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A case of prenatally diagnosed prune belly syndrome variant and congenital pouch colon in the United States: A case report 美国一例产前诊断为梅干-腹部综合征变异型和先天性袋状结肠的病例报告
Q4 Medicine Pub Date : 2021-09-25 DOI: 10.47338/jns.v10.1012
M. Yilma, Karen Trang, M. Schwab, M. Bowman, M. Sugi, J. Courtier, L. Baskin, D. Ozgediz
Background: Prune belly syndrome (PBS) and congenital pouch colon (CPC) are rare congenital syndromes with a low incidence in the United States (U.S.) with most CPC cases being from India. In this case report, we describe, to the best of our knowledge, the first PBS variant and CPC patient in the U.S.Case Presentation: A 30-year-old G2P0010 woman was referred to a tertiary center after an 18-week ultrasound showed a fetal abdominal mass. A prenatal MRI showed a dilated loop of bowel containing a mixture of urine and meconium, oligohydramnios, and a protuberant abdominal wall. Born at 37 weeks, the child’s physical exam was notable for a distended abdomen with thin abdominal musculature, non-palpable bilateral testes, no anal opening, and flat buttocks. Intra-operatively, a dilated cecum/ascending colon was noted with an abrupt change in caliber at the transverse colon, bilateral enlarged ureters, a left testis at the internal ring and no visualized right testis. A colostomy and mucous fistula were created 5 cm from the sigmoid pouch.Conclusion: While most reported cases of CPC undergo single stage repair (one operation) at 1 day of life, our patient underwent the first procedure of a staged repair at 16 hours of life given his clinical instability at the time as well as his unknown urological anatomy in the setting of urinary obstruction. This case demonstrates the importance of fetal imaging, multidisciplinary approach at a tertiary care center, and reinforces a staged repair when necessary.
背景:李子肚综合征(PBS)和先天性袋结肠(CPC)是美国罕见的先天性综合征,发病率低,大多数CPC病例来自印度。在本病例报告中,据我们所知,我们描述了美国第一例PBS变异和CPC患者。病例描述:一名30岁的G2P0010妇女在18周超声检查显示胎儿腹部肿块后被转至三级中心。产前MRI显示肠袢扩张,含有尿和胎便混合物,羊水过少,腹壁隆起。37周出生,体格检查发现腹部肿胀,腹部肌肉组织薄,双侧睾丸摸不到,无肛门开口,臀部扁平。术中可见盲肠/升结肠扩张,横结肠口径突变,双侧输尿管肿大,内环左侧睾丸,右侧睾丸未见。在距乙状结肠袋5cm处造结肠造口和粘液瘘。结论:大多数报道的CPC病例在出生后1天进行单阶段修复(一次手术),而我们的患者在出生后16小时进行了第一次分阶段修复手术,因为他当时的临床不稳定以及他在尿路梗阻背景下未知的泌尿解剖学。本病例证明了在三级护理中心进行胎儿成像和多学科治疗的重要性,并在必要时加强了分阶段修复。
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引用次数: 0
Meconium obstruction in a premature neonate: An etiology that may simulate spontaneous intestinal perforation 早产新生儿胎便梗阻:一种可能模拟自发性肠穿孔的病因
Q4 Medicine Pub Date : 2021-09-23 DOI: 10.47338/jns.v10.1013
James G. Glasser
A surgeon expects either necrotizing enterocolitis (NEC) or spontaneous intestinal perforation (SIP) in preemies with pneumoperitoneum. But there is another cause wherein the operative findings simulate meconium ileus [1]: The terminal ileum and colon are tiny and obstructed by inspissated meconium; The bowel proximal to the obstruction may be diffusely dilated, or there may be aneurysmal (segmental) dilatation, which may be single or multiple; Resection with ileostomy is all that is attempted in these tiny, fragile babies; Surprisingly, when the ileostomy is closed 4-6 weeks later, the bowel appears to be normal, the obstruction has cleared and the caliber disparity has disappeared, which suggests that immaturity was causative of the obstruction and the occasional aneurysmal dilatation.
一个外科医生预计坏死性小肠结肠炎(NEC)或自发性肠穿孔(SIP)在早产儿气腹。但手术表现酷似粪便性肠梗阻还有另一个原因:回肠末端和结肠细小,被密集的粪便阻塞;梗阻近端肠可呈弥漫性扩张,或动脉瘤状(节段性)扩张,可单发或多发;手术切除和回肠造口术是治疗这些娇小脆弱婴儿的唯一方法;令人惊讶的是,当4-6周后关闭回肠造口时,肠道看起来正常,阻塞已清除,口径差异消失,这表明不成熟是阻塞和偶尔的动脉瘤扩张的原因。
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引用次数: 0
Intraluminal pyloric duplication cyst- a rare cause of non-bilious vomiting in a neonate: A case report 幽门腔内重复囊肿——新生儿非胆汁性呕吐的罕见原因:一例报告
Q4 Medicine Pub Date : 2021-09-23 DOI: 10.47338/jns.v10.1006
Ravi Patcharu, Jaiprakash Yadav, K. Chand, Badal Parikh
Background: Duplications of the alimentary tract are rare congenital malformations, out of which, true pyloric duplications constitute only 2.2%. They present with non-bilious vomiting and mimic hypertrophic pyloric stenosis (HPS). Pyloric duplications that are intraluminal are not separately visible at laparotomy, making their diagnosis difficult. Case presentation: Our case is a neonate with an intraluminal pyloric duplication cyst who presented with recurrent vomiting. The radiological evaluation suggested a duplication cyst medial to the second part of the duodenum towards the stomach’s lesser curvature with features of gastric outlet obstruction. Intraoperatively, a cystic mass of 1 x 2 cm intraluminally was found on opening the pylorus which was excised and pyloroplasty was done. Postoperatively the baby was discharged in a stable condition.Conclusion: Intraluminal pyloric duplication cysts are rare and the clinical presentation mimics HPS. They should be considered as a differential diagnosis in a neonate presenting with features of gastric outlet obstruction.
背景:消化道重复是一种罕见的先天性畸形,其中真正的幽门重复仅占2.2%。它们表现为非胆汁性呕吐和类似肥厚性幽门狭窄(HPS)。腔内幽门重复在剖腹手术中不能单独看到,这使其诊断变得困难。病例介绍:我们的病例是一名新生儿,患有腔内幽门重复囊肿,并伴有反复呕吐。放射学评估显示,十二指肠第二部分内侧有一个朝向胃小曲的重复囊肿,具有胃出口梗阻的特征。术中,在打开幽门时发现管腔内1 x 2 cm的囊性肿块,将其切除并进行幽门成形术。术后婴儿出院,情况稳定。结论:腔内幽门重复囊肿是罕见的,临床表现类似HPS。对于以胃出口梗阻为特征的新生儿,应将其视为鉴别诊断。
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引用次数: 0
Cystic hygroma and esophageal atresia with tracheoesophageal fistula: Is there a genetic association? 囊性水肿和食管闭锁伴气管食管瘘:是否有遗传关系?
Q4 Medicine Pub Date : 2021-09-12 DOI: 10.47338/jns.v10.997
Sravanthi Vutukuru, N. Peters, S. Dogra, R. Samujh
Background: Tracheoesophageal fistula (TEF) is associated with many other congenital anomalies like cardiac, renal, and vertebral, but the association with cystic hygroma is very rare.Case Presentation: We report a neonate, antenatally diagnosed with cystic hygroma of the neck and incidentally diagnosed to have TEF. The baby was operated on after adequate stabilization and the cystic hygroma was managed with injection sclerotherapy. The genetic analysis for the FOX genes complex has been done in this case and the results showed no association between the two.Conclusion: There is a need for future studies to find out if the association between cystic hygroma and TEF is by chance or if any embryological or genetic cause can be attributed to it.
背景:气管食管瘘(TEF)与许多其他先天性异常有关,如心脏、肾脏和脊椎,但与囊性湿气瘤有关的情况非常罕见。病例介绍:我们报告了一名新生儿,出生前被诊断为颈部囊性湿瘤,偶然被诊断为TEF。婴儿在充分稳定后接受了手术,囊性湿瘤通过注射硬化治疗进行了治疗。在这种情况下,对FOX基因复合体进行了遗传分析,结果显示两者之间没有关联。结论:有必要在未来的研究中找出囊性湿气瘤和TEF之间的联系是否是偶然的,或者是否有任何胚胎或遗传原因可归因于此。
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引用次数: 0
Congenital segmental dilatation of intestine with different morphology: A case report 先天性不同形态节段性肠扩张1例
Q4 Medicine Pub Date : 2021-09-12 DOI: 10.47338/jns.v10.1021
Bilal Mirza, Sumaira Noor Maham, N. Talat
Background: Congenital segmental dilatation of intestine is a rare entity characterized by a localized dilated segment of the intestine. It manifests as neonatal intestinal obstruction. Occurrence with another surgical anomaly may masquerade it and complicate the treatment course.Case Presentation: A 1-day-old male neonate with anorectal malformation underwent colostomy as first stage of the treatment. The stoma did not function postoperatively. At repeat surgery, congenital segmental dilatation of the ileum, with slightly different morphology, was found. Resection of the lesion and ileostomy were formed. Postoperative course remained uneventful.Conclusion: Congenital segmental dilatation of intestine may be missed at initial surgery. Complete surgical resection is curative.
背景:先天性节段性肠扩张是一种罕见的以局部肠段扩张为特征的疾病。表现为新生儿肠梗阻。另一种手术异常可能会掩盖它,使治疗过程复杂化。病例介绍:1天大的男性新生儿肛门直肠畸形接受结肠造口术作为第一阶段的治疗。造口术后不起作用。在重复手术中,发现先天性回肠节段性扩张,形态略有不同。形成了病灶切除和回肠造口术。术后情况平静。结论:先天性节段性肠扩张术在初次手术时可能会错过。完全手术切除是可以治愈的。
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引用次数: 1
The Raveenthiran Syndrome—Ectopic testis within Spigelian hernia: Few additional remarks Raveenthiran综合征——Spigelian疝内异位睾丸:几点补充意见
Q4 Medicine Pub Date : 2021-09-09 DOI: 10.47338/jns.v10.1024
Venkatachalam Raveenthiran
I read, with great interest, the article by Sinacer et al. on the association of congenital Spigelian hernia with cryptorchidism, polydactyly, and anal stenosis.[1] Although this association is known since 1895, only in 2005 it was recognized as a new syndrome.[2,3] Since then there has been an increasing awareness about this clinical entity.[4] In 2015 Moles-Morenilla et al. named it ‘Raveenthiran syndrome’ and classified it into two types: type-1 is an exclusive association of Spigelian hernia and ectopic testis while type-2 is Spigelian hernia and ectopic testis associated with other anomalies.[5] Accordingly, the newborn reported by Sinacer et al. belongs to type-2 Raveenthiran syndrome.
我怀着极大的兴趣阅读了Sinacer等人关于先天性Spigelian疝与隐睾、多指畸形和肛门狭窄之间关系的文章虽然这种关联早在1895年就已为人所知,但直到2005年才被认为是一种新的综合征。[2,3]从那时起,人们对这一临床实体的认识越来越高2015年Moles-Morenilla等人将其命名为“Raveenthiran综合征”,并将其分为两种类型:1型是Spigelian疝和异位睾丸的独家关联,2型是Spigelian疝和异位睾丸合并其他异常据此,Sinacer等报道的新生儿属于2型Raveenthiran综合征。
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引用次数: 0
Type III wide colonic atresia: An etiological introspection III型宽结肠闭锁的病因反思
Q4 Medicine Pub Date : 2021-08-23 DOI: 10.47338/jns.v10.1020
M. Hassan
{"title":"Type III wide colonic atresia: An etiological introspection","authors":"M. Hassan","doi":"10.47338/jns.v10.1020","DOIUrl":"https://doi.org/10.47338/jns.v10.1020","url":null,"abstract":"","PeriodicalId":34201,"journal":{"name":"Journal of Neonatal Surgery","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2021-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"47475769","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: 1
Congenital Spigelian hernia in a neonate associated with several anomalies: A case report 新生儿先天性Spigelian疝合并几种异常:1例报告
Q4 Medicine Pub Date : 2021-08-13 DOI: 10.47338/jns.v10.972
Samira Sinacer, Bilal Zakaria Semari, Soumia Khemari, Ahlem Kharchi, Assia Haif, Zineddine Soualili
Background: Spigelian hernia (SH) is a rare entity characterized by a defect of the anterior abdominal wall located along the Spigelian line, it may be congenital or acquired.Association with other anomalies is worth reporting.Case Presentation: A 22-day-old male newborn was admitted with strangulated right inguinal hernia and operated on emergently. Clinical examination also revealed a Spigelian hernia in the left lower abdominal quadrant with bilateral cryptorchidism, associated with polydactyly of the small right finger and anal stenosis. At surgery, the SH contained a part of the small intestine and the ipsilateral undescended testis.Conclusion: Pediatric SH is rare, but its association with undescended ipsilateral testis is frequent. Other abnormalities can be concomitant to this association.
背景:Spigelian hernia (SH)是一种罕见的实体,其特征是沿Spigelian线的前腹壁缺陷,可能是先天性的或获得性的。与其他异常的关联值得报告。病例介绍:一名22日出生的男婴因绞窄性右腹股沟疝入院接受急诊手术。临床检查还发现左下腹Spigelian疝伴双侧隐睾,伴右小指多指畸形和肛门狭窄。手术时,SH包含部分小肠和同侧隐睾。结论:小儿SH是罕见的,但其与同侧睾丸未下降是常见的。其他异常可伴随这种关联。
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引用次数: 1
Outcomes following the establishment of Qatar’s quaternary neonatal general surgical service in a hospital with a “Tabula Rasa” for service development 在一家拥有“Tabula Rasa”的医院建立卡塔尔四级新生儿普通外科服务后的结果
Q4 Medicine Pub Date : 2021-08-09 DOI: 10.47338/jns.v10.975
B. Khalil, Abdalla E. Zarroug, Abubakr Elawad, S. Moussa, C. Donnelly, Saleem Mammoo, G. Brisseau, Alison R Bedford Russell, H. Hummler
Background: Sidra Medicine is the first dedicated Children and Maternity hospital in the State of Qatar. This paper describes the preparations for and results of activating quaternary neonatal surgical services in a brand new, "greenfield" hospital. We believe that these are the first published national results of neonatal general surgical services from the Gulf region.Methods: A review of surgical babies below four weeks corrected age from 1st of April 2018 – 31st of March 2020 was undertaken. Patient demographics, primary diagnosis, surgical procedures, 30-day postoperative mortality, overall mortality, and cause of death were recorded.Results: One hundred and sixty-nine babies (169) were identified (44.4% term and 55.6% preterm). Major surgery included laparotomy (76), congenital diaphragmatic hernia repair (20), surgery for anorectal malformations (25), and esophageal atresia (13). One set of conjoined twins were also separated successfully.  Fourteen babies died, resulting in overall mortality of 8.3 %. Excluding babies who died of life-limiting conditions, two babies died within 30 days of surgery, resulting in 30 days postoperative mortality rate of 1.2 %.Conclusions: The neonatal surgical mortality rate is comparable with those from top international centers. This low rate could be attributed to the high level of expertise preceded by months of preparations.
背景:Sidra Medicine是卡塔尔国第一家专门的儿童和产妇医院。本文介绍了在一家全新的“绿地”医院启用四级新生儿外科服务的准备工作和结果。我们相信,这是海湾地区首次公布的新生儿普通外科服务的全国结果。方法:对2018年4月1日至2020年3月31日4周以下矫正年龄的外科婴儿进行回顾性研究。记录患者人口统计、初步诊断、外科手术、术后30天死亡率、总死亡率和死因。结果:共确认169名婴儿(44.4%足月,55.6%早产)。主要手术包括剖腹手术(76)、先天性膈疝修补术(20)、肛门直肠畸形手术(25)和食道闭锁手术(13)。一对连体双胞胎也成功分离。14名婴儿死亡,总死亡率为8.3%。不包括因生命受限而死亡的婴儿,有两名婴儿在手术后30天内死亡,导致术后30天死亡率为1.2%。结论:新生儿手术死亡率与国际顶级中心的死亡率相当。这一低比率可归因于此前数月的准备工作所具备的高水平专业知识。
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引用次数: 0
Jejunal atresia with ileojejunal knotting: An unusual presentation 空肠闭锁伴回空肠结:一种不寻常的表现
Q4 Medicine Pub Date : 2021-07-23 DOI: 10.47338/JNS.V10.951
N. Pawar, Pramila Sharma, P. Mittal, V. Chaturvedi
      
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引用次数: 0
期刊
Journal of Neonatal Surgery
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