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Pleural effusion after Nuss procedure: a case series Nuss手术后胸腔积液:一个病例系列
IF 0.2 Q4 PEDIATRICS Pub Date : 2025-10-01 Epub Date: 2025-08-13 DOI: 10.1016/j.epsc.2025.103086
Hendrik van Braak , Suzanne W.J. Terheggen-Lagro , Joël Israels , Joost van Schuppen , Eline E. Deurloo , Justin R. de Jong

Introduction

Pleural effusion is an underreported and poorly understood complication of the Nuss procedure.

Cases presentation

Case 1: A 16-year-old female presented five weeks postoperatively with dyspnea (CRP 258 mg/L), left-sided pleural and pericardial effusion. Drainage and treatment with amoxicillin/clavulanic acid, gentamicin, diuretics, ibuprofen, and prednisolone led to improvement. After steroid discontinuation, effusion recurred despite treatment with amoxicillin/clavulanic acid and gentamicin. Cultures remained negative. She required thoracic drainage and received oral clindamycin, later switched to ciprofloxacin, with eventual recovery.
Case 2: A 17-year-old male presented with right-sided chest pain and pleural effusion two weeks after a complicated Nuss procedure (due to extensive adhesiolysis for pneumonia-related lung adhesions. Despite antibiotics, symptoms progressed, and he was admitted (CRP 240 mg/L). Chest drainage yielded 1300 mL clear fluid; cultures were negative. Recurrent effusion eventually required video-assisted thoracoscopic surgery. He was treated with amoxicillin/clavulanic acid after surgery.
Case 3: A 14-year-old female developed fever one week postoperatively (CRP 123 mg/L), with no abnormalities on imaging. Three weeks later, she developed right-sided effusion; 600 mL was drained, and she received ceftriaxone and clindamycin. Recurrence occurred within two weeks; oral prednisolone was added, resulting in rapid improvement. She was discharged on cotrimoxazole and tapered steroids. Two months later, imaging for persistent fatigue revealed Nuss bar dislocation and pneumothorax, requiring bar repositioning.

Conclusion

Pleural effusion after the Nuss procedure can be caused by a reactive inflammatory response, triggered by pleural or mechanical irritation. If an infection and bar displacement are ruled out, a course of corticosteroid therapy may be effective.
胸腔积液是Nuss手术的一种未被充分报道和理解的并发症。病例1:16岁女性,术后5周出现呼吸困难(CRP 258mg /L),左侧胸膜及心包积液。引流和阿莫西林/克拉维酸、庆大霉素、利尿剂、布洛芬和强的松龙治疗导致改善。停用类固醇后,尽管用阿莫西林/克拉维酸和庆大霉素治疗,积液仍复发。文化仍然是消极的。她需要胸腔引流并口服克林霉素,后来改用环丙沙星,最终恢复。病例2:一名17岁男性患者在接受复杂的Nuss手术两周后出现右侧胸痛和胸腔积液(由于肺炎相关肺粘连的广泛粘连溶解)。尽管使用了抗生素,但症状仍有进展,患者入院(CRP 240 mg/L)。胸腔引流清液1300 mL;文化是消极的。复发性积液最终需要电视胸腔镜手术。术后给予阿莫西林/克拉维酸治疗。病例3:14岁女性术后1周出现发热(CRP 123mg /L),影像学未见异常。三周后,她出现右侧积液;引流600ml,给予头孢曲松和克林霉素治疗。两周内复发;口服强的松龙后,病情迅速好转。她出院时服用复方新诺明和减量类固醇。两个月后,持续性疲劳影像学显示努斯棒脱位和气胸,需要重新定位棒。结论Nuss手术后胸腔积液可由胸膜或机械刺激引起的反应性炎症反应引起。如果排除感染和棒移位,一个疗程的皮质类固醇治疗可能是有效的。
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引用次数: 0
Suprapubic dermoid sinus in a 9-month-old infant: A case report 9个月婴儿耻骨上皮样窦1例
IF 0.2 Q4 PEDIATRICS Pub Date : 2025-10-01 Epub Date: 2025-07-14 DOI: 10.1016/j.epsc.2025.103058
Eiman Yassir Musa Hussain , Tarek Abdelazeem Sabra , Sarah Magdy Abdelmohsen

Introduction

Suprapubic dermoid sinuses are rare congenital anomalies resulting from incomplete ectodermal separation during embryogenesis.

Case presentation

A 9-month-old male infant presented with a midline suprapubic dimple noted since birth and no other medical history. Ultrasound revealed a superficial sinus tract, and Magnetic Resonance Imaging (MRI) confirmed a well-defined, blind-ending tract without any deeper communication. A complete excision was done in the operating room under general anesthesia. We guided the dissection of the sinus with methylene blue injected in it. The histopathology confirmed the diagnosis of a dermoid sinus lined with keratinized squamous epithelium containing dermal appendages. The postoperative recovery was uneventful. No recurrence was observed at the 1-month, 3-month, and 6-month follow-up visits.

Conclusion

Dermoid sinuses, although rare, should be included in the differential diagnosis of midline congenital dimples in infants.
耻骨上皮样窦是一种罕见的先天性畸形,是由胚胎发生时外胚层分离不完全引起的。病例介绍:一名9个月大的男婴,自出生以来就有耻骨上中线酒窝,无其他病史。超声显示浅表窦道,磁共振成像(MRI)证实了一个明确的,无任何深层沟通的盲尾窦道。在全身麻醉下在手术室进行了完全切除。我们在鼻窦内注入亚甲基蓝,引导鼻窦剥离。组织病理学证实了皮样窦内衬角质化鳞状上皮,含有真皮附属物。术后恢复顺利。随访1个月、3个月、6个月均无复发。结论皮样窦虽少见,但应作为婴儿先天性中线酒窝的鉴别诊断之一。
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引用次数: 0
Imperforate anus, multiple intestinal atresia and unilateral facial hypoplasia: A case series 肛门闭锁、多发性肠闭锁及单侧面部发育不全1例
IF 0.2 Q4 PEDIATRICS Pub Date : 2025-10-01 Epub Date: 2025-07-30 DOI: 10.1016/j.epsc.2025.103076
Joy Ayemoba , Carmelle Romain , Kathryn J. Rowland

Introduction

Imperforate anus and intestinal atresia are rare but well understood congenital disorders which affect the alimentary tract. Like intestinal atresia, facial hypoplasia is another congenital anomaly likely secondary to a vascular injury in utero.

Case presentation

Case 1 discusses a female born at 32 weeks with a prenatal diagnosis of duodenal atresia and discovery of imperforate anus and unilateral maxillary hypoplasia at birth. She was emergently taken to the operating room for bowel obstruction. During that operation, she underwent repair of multiple segments of atresia, a duodenoduodenostomy, and creation of an end ileostomy and mucus fistula. At two months of age, she returned to the operating room to restore continuity of the distal atretic bowel. At six months old she presented with a partial bowel obstruction secondary to a jejunal duplication cyst. Case 2 discusses a full-term male presenting with unilateral facial hypoplasia and imperforate anus at birth. Given failure to pass meconium and worsening abdominal distension he was taken to surgery, where multiple segments of intestinal atresia were identified. During this operation he was left in discontinuity following creation of a diverting loop ileostomy and colostomy. Two months later, he underwent repeat exploratory laparotomy during which multiple segments of atretic bowel were repaired. At four months of age, he underwent reversal of the diverting loop ileostomy followed by completion of posterior sagittal anorectoplasty at nine months old.

Conclusion

Intestinal atresias should be highly suspected in patients who present with anorectal malformations in conjunction with facial hypoplasia.
肛门闭锁和肠道闭锁是一种罕见的影响消化道的先天性疾病。像肠闭锁一样,面部发育不全是另一种先天性异常,可能继发于子宫内的血管损伤。病例1讨论了一名32周出生的女性,产前诊断为十二指肠闭锁,出生时发现肛门闭锁和单侧上颌发育不全。她因肠梗阻被紧急送往手术室。在手术中,她接受了多段闭锁修复术、十二指肠吻合术、回肠末端造口术和粘液瘘术。两个月大时,她回到手术室恢复远端锁肠的连续性。在6个月大时,她表现为继发于空肠重复囊肿的部分肠梗阻。病例2讨论了一个足月男性在出生时表现为单侧面部发育不全和肛门闭锁。由于未能排出胎便,腹胀加剧,他被送往手术,在那里发现了多段肠闭锁。在手术中,他在创建一个转移袢回肠造口术和结肠造口术后处于不连续状态。两个月后,他再次进行探查性剖腹手术,修复了多段闭锁肠。在4个月大时,他接受了回肠转袢造口术的逆转,并在9个月大时完成了后矢状肛肠成形术。结论肛肠畸形合并面部发育不全患者应高度警惕肠闭锁。
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引用次数: 0
Type IVA choledochal cyst with gallbladder duplication: a case report IVA型胆总管囊肿伴胆囊重复1例
IF 0.2 Q4 PEDIATRICS Pub Date : 2025-10-01 Epub Date: 2025-08-25 DOI: 10.1016/j.epsc.2025.103096
Saalim Nazki , Saswati Behera , Akshat Sudhanshu

Background

Gallbladder duplication is a rare congenital anomaly, and its coexistence with choledochal cysts is exceptionally uncommon.

Case

A 7-year-old female presented with recurrent right upper quadrant abdominal pain over several months. Laboratory investigations revealed elevated serum AST (142 IU/L) (normal range 8–33 IU/L) and ALT (158 IU/L) (normal range 4–36 IU/L), with mildly raised alkaline phosphatase; bilirubin levels were within normal limits. Abdominal ultrasonography (USG) demonstrated common bile duct dilation, and Magnetic Resonance Cholangiopancreatography (MRCP) confirmed a Type IVA choledochal cyst. Laparoscopic excision of the cyst with hepaticoduodenostomy was planned. Intraoperatively, a V shaped duplicated intrahepatic bilobed gallbladder was discovered, with a single cystic artery and a single cystic duct (bifurcated high up distally to drain the duplicated lobes of gall bladder) draining into the common bile duct. Both gallbladders were excised, and a hepaticoduodenostomy was performed. Histopathology confirmed gallbladder duplication with chronic inflammatory changes and no dysplasia.

Conclusion

Gallbladder duplication is a rare congenital anomaly that may not be identified on preoperative imaging, particularly in the setting of complex biliary anomalies like choledochal cyst.
背景:胆囊复制是一种罕见的先天性异常,其与胆总管囊肿共存是非常罕见的。病例1:7岁女性,反复出现右上腹疼痛数月。实验室检查显示血清AST升高(142 IU/L)(正常范围8-33 IU/L)和ALT升高(158 IU/L)(正常范围4-36 IU/L),碱性磷酸酶轻度升高;胆红素水平在正常范围内。腹部超声(USG)显示胆总管扩张,磁共振胆管胰胆管造影(MRCP)证实IVA型胆总管囊肿。计划行腹腔镜囊肿切除并肝十二指肠切开术。术中发现一V型复制肝内双叶胆囊,单根囊性动脉和单根囊性导管(向上分叉以排出复制的胆囊叶)汇入总胆管。切除两个胆囊,并行肝十二指肠切开术。组织病理学证实胆囊复制伴慢性炎症改变,无发育不良。结论全膀胱重复是一种罕见的先天性异常,术前影像学检查难以发现,尤其是胆总管囊肿等复杂的胆道异常。
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引用次数: 0
Early-onset infantile hypertrophic pyloric stenosis: a case report 早发性婴儿肥厚性幽门狭窄1例
IF 0.2 Q4 PEDIATRICS Pub Date : 2025-10-01 Epub Date: 2025-08-17 DOI: 10.1016/j.epsc.2025.103090
Eden Belay Tilahun , Tihitena Nigussie mamo , Werkneh Demissie Hailemariam , Gobena Mormata Dima , Nardos Mulu Admasu , Abraham Sisay Abie

Introduction

Infantile hypertrophic pyloric stenosis (IHPS) is a common cause of non-bilious projectile vomiting in infants, typically presenting between 4 and 8 weeks of age. Early-onset cases, particularly those occurring within the first few days of life, are rare and often challenging to diagnose due to lack of standardized sonographic criteria for this age group.

Case presentation

A 12-day-old male neonate presented with persistent non-bilious projectile vomiting beginning on the first days of life. He was born at full term via spontaneous vaginal delivery to a 26-year-old primigravida mother after an uncomplicated pregnancy. Initial evaluation at a local health center led to conservative management for presumed gastroesophageal reflux. Persisting symptoms prompted referral after an initial ultrasound showed a pyloric muscle thickness of 3.6 mm and a length of 16 mm, suggestive of IHPS. However, due to the uncommon occurrence of IHPS at such an early age, a repeat ultrasound was done, which was interpreted as normal. An upper gastrointestinal contrast study was done to rule out intestinal malrotation and other sources of gastric outlet obstruction. Due to the persistence of symptoms, a third ultrasound was done on day of life 21, which showed a pyloric length of 16.6 mm and a thickness of 4.2 mm, conclusive for IHPS. The patient underwent an open Ramstedt pyloromyotomy without complications. The postoperative recovery was complicated by a hospital-acquired pneumonia, which was successfully treated. The patient was discharged on postoperative day 16 and remained asymptomatic at six months of follow-up, with normal growth and development.

Conclusion

Pyloric stenosis rarely occurs within the first few weeks of life but should be included in the differential diagnosis of newborns who develop persistent projectile non-bilious vomiting.
婴儿肥厚性幽门狭窄(IHPS)是婴儿非胆汁性抛射性呕吐的常见原因,通常出现在4至8周龄之间。早发病例,特别是那些发生在生命最初几天的病例,是罕见的,而且由于缺乏标准化的超声检查标准,往往难以诊断。病例介绍:一名12天大的男婴在出生的第一天就出现了持续的非胆汁性抛射性呕吐。他是一位26岁的初产妇在顺利怀孕后通过阴道自然分娩足月出生的。在当地卫生中心进行的初步评估导致了对疑似胃食管反流的保守治疗。最初的超声检查显示幽门肌厚度3.6 mm,长度16 mm,提示IHPS,持续症状促使转诊。然而,由于IHPS在这么小的年龄很少发生,因此再次做了超声检查,解释为正常。进行了上消化道对比研究,以排除肠道旋转不良和其他胃出口梗阻的来源。由于症状持续存在,在第21天进行了第三次超声检查,显示幽门长度为16.6 mm,厚度为4.2 mm,确定为IHPS。患者接受了开放式Ramstedt幽门肌切开术,无并发症。术后恢复因医院获得性肺炎而变得复杂,但已成功治疗。患者术后第16天出院,随访6个月无症状,生长发育正常。结论幽门狭窄很少发生在出生后几周内,但应列入新生儿持续性射性非胆汁性呕吐的鉴别诊断。
{"title":"Early-onset infantile hypertrophic pyloric stenosis: a case report","authors":"Eden Belay Tilahun ,&nbsp;Tihitena Nigussie mamo ,&nbsp;Werkneh Demissie Hailemariam ,&nbsp;Gobena Mormata Dima ,&nbsp;Nardos Mulu Admasu ,&nbsp;Abraham Sisay Abie","doi":"10.1016/j.epsc.2025.103090","DOIUrl":"10.1016/j.epsc.2025.103090","url":null,"abstract":"<div><h3>Introduction</h3><div>Infantile hypertrophic pyloric stenosis (IHPS) is a common cause of non-bilious projectile vomiting in infants, typically presenting between 4 and 8 weeks of age. Early-onset cases, particularly those occurring within the first few days of life, are rare and often challenging to diagnose due to lack of standardized sonographic criteria for this age group.</div></div><div><h3>Case presentation</h3><div>A 12-day-old male neonate presented with persistent non-bilious projectile vomiting beginning on the first days of life. He was born at full term via spontaneous vaginal delivery to a 26-year-old primigravida mother after an uncomplicated pregnancy. Initial evaluation at a local health center led to conservative management for presumed gastroesophageal reflux. Persisting symptoms prompted referral after an initial ultrasound showed a pyloric muscle thickness of 3.6 mm and a length of 16 mm, suggestive of IHPS. However, due to the uncommon occurrence of IHPS at such an early age, a repeat ultrasound was done, which was interpreted as normal. An upper gastrointestinal contrast study was done to rule out intestinal malrotation and other sources of gastric outlet obstruction. Due to the persistence of symptoms, a third ultrasound was done on day of life 21, which showed a pyloric length of 16.6 mm and a thickness of 4.2 mm, conclusive for IHPS. The patient underwent an open Ramstedt pyloromyotomy without complications. The postoperative recovery was complicated by a hospital-acquired pneumonia, which was successfully treated. The patient was discharged on postoperative day 16 and remained asymptomatic at six months of follow-up, with normal growth and development.</div></div><div><h3>Conclusion</h3><div>Pyloric stenosis rarely occurs within the first few weeks of life but should be included in the differential diagnosis of newborns who develop persistent projectile non-bilious vomiting.</div></div>","PeriodicalId":45641,"journal":{"name":"Journal of Pediatric Surgery Case Reports","volume":"121 ","pages":"Article 103090"},"PeriodicalIF":0.2,"publicationDate":"2025-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144863573","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
Familial intestinal malrotation and cleft lip and palate: A case report 家族性肠旋转不良并发唇腭裂1例
IF 0.2 Q4 PEDIATRICS Pub Date : 2025-10-01 Epub Date: 2025-07-28 DOI: 10.1016/j.epsc.2025.103072
Kamela Loo , Krista Lai , Vidhi Shah

Introduction

The etiology of intestinal malrotation is unknown and it can occur in isolation or with other congenital anomalies. Although case reports have reported genetic etiologies of intestinal malrotation, association with cleft lip and palate is rare.

Case presentation

A 37 1/7-week male infant was born via C-section with a birth weight of 3270g and a prenatal diagnosis of cleft lip and palate. His Apgar scores at 1 and 5 minutes were 7 and 8, respectively, and he briefly required continuous positive airway pressure in the delivery room before weaning to room air. The infant was admitted to the neonatal intensive care unit for hypoglycemia and poor feeding. On day of life twelve, he developed bloody stools, metabolic acidosis, and abdominal distention, which progressed to respiratory failure, coagulopathy, and hypovolemic shock. Abdominal x-rays demonstrated a stomach bubble with paucity of distal gas. An abdominal ultrasound demonstrated inversion of the superior mesenteric vein and artery, which was highly suggestive of an intestinal malrotation with midgut volvulus. The patient underwent an emergent Ladd procedure without bowel resection. He had an uneventful postoperative recovery, with progression to full enteral feedings. The patient's older half sibling also had cleft lip and palate, experienced recurrent vomiting, and was diagnosed with malrotation requiring an elective Ladd procedure.

Conclusion

This case study suggests that the association of cleft lip and palate and intestinal malrotation may have a genetic background. Newborns who have cleft lip and palate who have siblings with this association should undergo imaging studies to rule out intestinal malrotation.
肠道旋转不良的病因尚不清楚,它可以单独发生或与其他先天性异常一起发生。虽然病例报告已经报道了肠道旋转不良的遗传病因,但与唇腭裂的关联是罕见的。一例37又1/7周的男婴经剖腹产出生,出生体重3270g,产前诊断为唇腭裂。他在第1分钟和第5分钟的Apgar评分分别为7分和8分,在断奶到室内空气之前,他在产房短暂地需要持续气道正压。该婴儿因低血糖和喂养不良被送入新生儿重症监护病房。第12天,患者出现便血、代谢性酸中毒、腹胀,并发展为呼吸衰竭、凝血功能障碍和低血容量性休克。腹部x光片显示胃泡伴远端气体缺乏。腹部超声显示肠系膜上静脉和动脉倒置,高度提示肠道旋转不良伴中肠扭转。患者接受了紧急Ladd手术,但未切除肠道。他术后恢复顺利,并进展到完全肠内喂养。患者同父异母的哥哥姐姐也患有唇腭裂,反复呕吐,并被诊断为旋转不良,需要选择性Ladd手术。结论本病例提示唇腭裂与肠道旋转不良的关联可能具有遗传背景。唇腭裂的新生儿如果有兄弟姐妹有这种关联,应接受影像学检查以排除肠道旋转不良。
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引用次数: 0
Spontaneous closure of a patent processus vaginalis: a case report 阴道未闭自发性闭合1例报告
IF 0.2 Q4 PEDIATRICS Pub Date : 2025-10-01 Epub Date: 2025-08-11 DOI: 10.1016/j.epsc.2025.103084
Haley Kittle , Amber Lee Geng , Loren Berman , Erin Teeple

Introduction

Patent process vaginalis and inguinal hernia are prolific in infants, and their repair is a common pediatric surgical procedure. However, there is a paucity of direct evidence detailing the spontaneous closure of patent processus vaginalis.

Case presentation

A 4-month-old boy born prematurely at 34-weeks with low gestational birth weight, underwent laparoscopic gastrostomy tube placement for oropharyngeal dysphagia and was incidentally found to have bilateral patent processes vaginalis. Due to his lack of symptoms, he was managed under the watchful waiting approach. His parents were educated about signs and symptoms of inguinal hernia and were instructed to contact the team with any concerns. He represented at 6 years of age with symptomatic right-sided inguinal hernia, and during planned laparoscopic surgical repair, he was found to have spontaneous closure of the processus vaginalis on the left side. His right sided hernia was repaired, and his left side was left untouched. The patient tolerated the procedure well and has had no signs nor symptoms of right sided recurrence nor new onset of left sided hernia to date. He will follow up as needed if there are any concerns for hernia on the left side.

Conclusion

Patent processus vaginalis may progress to symptomatic inguinal hernia or may close spontaneously. Patients with bilateral patent processus vaginalis may exhibit a different outcome on each side.
腹股沟疝和阴道未平突疝在婴儿中发病率很高,其修复是一种常见的儿科外科手术。然而,缺乏直接证据详细说明阴道未闭的自发关闭。一例34周早产4个月男婴,出生体重低,因口咽吞咽困难行腹腔镜胃造口管置入术,偶然发现双侧阴道未闭。由于他没有症状,所以采取了观察等待的方法。他的父母被告知腹股沟疝的体征和症状,并被指示与团队联系任何担忧。他在6岁时表现为有症状的右侧腹股沟疝,在计划的腹腔镜手术修复中,他被发现左侧阴道突自发关闭。他的右侧疝气被修复,而他的左侧没有受到影响。患者对手术的耐受性良好,到目前为止没有出现右侧复发的症状和体征,也没有出现左侧疝的新发。如果有任何关于左侧疝气的担忧,他将会跟进检查。结论阴道突未闭可发展为症状性腹股沟疝或自行闭合。双侧阴道突未闭的患者可能会表现出不同的结果。
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引用次数: 0
Three-dimensional modelling and printing for preoperative planning in the resection of an ischiorectal epithelioid sarcoma: a case report 三维建模和打印用于坐骨直肠上皮样肉瘤切除术的术前规划:1例报告
IF 0.2 Q4 PEDIATRICS Pub Date : 2025-10-01 Epub Date: 2025-07-30 DOI: 10.1016/j.epsc.2025.103079
Rachel J. Livergant , Trevor D. Hamilton , Daniel G. Rosenbaum , Sima Zakani , John Jacob , Shahrzad Joharifard

Introduction

Epithelioid sarcomas are rare malignant mesenchymal tumours, accounting for approximately 3–4 % of pediatric soft-tissue sarcomas, and present unique surgical challenges due to their infiltrative nature and proximity to critical anatomical structures. While three-dimensional modelling has emerged as a valuable adjunct in preoperative planning for complex tumours, its use in pediatric soft-tissue sarcomas is rare, with no described cases involving epithelioid sarcomas.

Case presentation

A 13-year-old girl presented with a year-long history of perineal pain and a palpable mass. Imaging revealed a tumour involving the rectum and external sphincter, abutting the posterior vaginal wall. Biopsy confirmed epithelioid sarcoma and staging showed indeterminate pulmonary nodules. She underwent neoadjuvant chemoradiotherapy. Two surgical options were considered: abdominoperineal resection with hysterectomy or vaginal preservation with increased risk of residual disease. A three-dimensional model with 2cm margins was created to aid decision-making. After multidisciplinary review and model consultation, the patient opted for vaginal preservation. She underwent abdominoperineal resection with VRAM flap reconstruction. Final pathology confirmed negative margins. Nearly three years later, she developed a pancreatic tail metastasis and progressive pulmonary lesions, which were treated with distal pancreatectomy, splenectomy, radiotherapy, and cryoablation. Additional metastases in the stomach, scalp, retroperitoneum, and flank are being managed with palliative radiotherapy.

Conclusion

Three-dimensional printed models can be helpful in the preoperative planning for the resection of tumours of the ischiorectal region.
上皮样肉瘤是一种罕见的恶性间充质肿瘤,约占小儿软组织肉瘤的3 - 4%,由于其浸润性和靠近关键解剖结构,给手术带来了独特的挑战。虽然三维建模在复杂肿瘤的术前规划中已经成为一种有价值的辅助手段,但它在小儿软组织肉瘤中的应用很少见,没有涉及上皮样肉瘤的病例。病例介绍:一名13岁的女孩,因会阴疼痛和可触及的肿块长达一年。影像显示肿瘤累及直肠及外括约肌,紧靠阴道后壁。活检证实为上皮样肉瘤,分期不确定为肺结节。她接受了新辅助放化疗。考虑了两种手术选择:腹部会阴切除合并子宫切除术或阴道保留,但会增加残留疾病的风险。为了帮助决策,他们创建了一个边缘为2厘米的三维模型。经过多学科回顾和模型咨询,患者选择阴道保留。她接受了腹部会阴切除术和VRAM皮瓣重建。最终病理证实阴性边缘。近三年后,她出现胰腺尾部转移和进行性肺部病变,并接受远端胰腺切除术、脾切除术、放疗和冷冻消融治疗。胃、头皮、腹膜后和腹部的其他转移灶正在接受姑息性放疗。结论三维打印模型有助于坐骨直肠肿瘤切除术的术前规划。
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引用次数: 0
Congenital megacalycosis managed with observation only: A case report 仅观察治疗先天性巨核症1例
IF 0.2 Q4 PEDIATRICS Pub Date : 2025-10-01 Epub Date: 2025-08-20 DOI: 10.1016/j.epsc.2025.103082
Kristy Alexandra Smith, Mohamed Sameh Shalaby

Introduction

Congenital megacalycosis (CM) is one of the rarest congenital renal anomalies. It typically presents with significant calyceal dilatation resembling severe pelvi-ureteric junction obstruction (PUJO) in a kidney with an intra-renal pelvis. Normal drainage on mercaptoacetyltriglycine (MAG3) renogram confirms the CM diagnosis.

Case presentation

A male infant presented antenatally with significant unilateral calyceal dilatation and presumed diagnosis of severe PUJO. Growth scans at 32, 36 and 39 weeks showed an increasingly dilated renal pelvis with normal liquor volume. He was delivered at 39 weeks by emergency caesarean due to fetal distress. Postnatal ultrasound (USS) was consistent with the antenatal scans with an anteroposterior diameter (APD) of 21mm but a MAG3 showed normal drainage and equal split function, confirming the diagnosis of megacalycosis rather than classic PUJO. The patient therefore avoided surgery and has been managed with active observation with 3 monthly USS. Repeat USS at 15 months shows improvement in dilatation (APD 13mm), and no deterioration of drainage or drop of function on repeat MAG3 at 12 months.

Conclusion

Congenital megacalycosis should be ruled out with MAG3 in children with suspected pelviureteric junction obstruction as the two conditions may have a similar USS appearance but have a significantly different management strategy.
摘要先天性巨alysis (CM)是一种罕见的先天性肾脏异常。典型表现为肾盏扩张,类似于肾盂-输尿管交界处严重梗阻(PUJO)。巯基乙酰甘油三酯(MAG3)肾图引流正常,证实CM诊断。一个男婴产前表现为明显单侧肾盏扩张,推测诊断为严重的PUJO。32、36和39周的生长扫描显示肾盂逐渐扩张,但尿量正常。由于胎儿窘迫,他在39周时通过紧急剖腹产分娩。产后超声(USS)与产前扫描一致,前后径(APD)为21mm,但MAG3显示引流正常,分裂功能均等,确认诊断为巨结石而不是典型的PUJO。因此,患者避免了手术,并进行了3个月的USS积极观察。重复号在15个月显示改善膨胀(adp 13毫米),也没有恶化的排水或删除功能重复MAG3在12个月。结论怀疑肾盂输尿管连接处梗阻的儿童,应排除先天性巨结石,因为这两种情况可能具有相似的USS外观,但处理策略有明显不同。
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引用次数: 0
Omphalomesenteric duct cyst containing ectopic gastric mucosa in a toddler: a case report 幼儿含胃粘膜异位的脐肠管囊肿1例报告
IF 0.2 Q4 PEDIATRICS Pub Date : 2025-10-01 Epub Date: 2025-09-03 DOI: 10.1016/j.epsc.2025.103099
Shital Bhaisare , Jayakumar T K , Harshal Madavi , Kiran Khedkar , Nisha Meshram

Introduction

Omphalomesenteric duct cyst (OMDC) is an uncommon remnant of the omphalomesenteric duct. The occurrence of ectopic gastric mucosa within such cysts is rare and may lead to complications due to acid secretion.

Case presentation

A 2-year-old girl presented to us with persistent serous umbilical discharge since birth, associated with severe periumbilical excoriation. Ultrasonography showed a 1-cm cystic lesion beneath the umbilicus. Umbilical exploration revealed a cyst posterior to the umbilicus in the pre-peritoneal space, communicating with the skin through a fine sinus tract. The cyst was excised completely. Histopathological examination demonstrated ectopic gastric mucosa within the cyst wall. Postoperatively, the child had an uneventful recovery and was discharged home the day after the procedure. She remains asymptomatic and the excoriation of the skin has resolved.

Conclusion

Children presenting with persistent umbilical discharge accompanied by severe skin excoriation should be evaluated for an omphalomesenteric duct cyst containing ectopic gastric mucosa.
摘要肛管囊肿(OMDC)是一种罕见的肛管残余病变。这种囊肿内发生胃粘膜异位是罕见的,并可能因胃酸分泌而导致并发症。病例介绍:一名2岁女童自出生以来持续出现浆液性脐带分泌物,并伴有严重的脐周擦伤。超声示脐下1厘米囊性病变。脐部探查发现一个囊肿位于脐后腹膜前间隙,通过细小的窦道与皮肤相通。囊肿被完全切除了。组织病理学检查显示囊肿壁内胃粘膜异位。术后,患儿顺利康复,并于术后第二天出院回家。她仍无症状,皮肤擦伤已消失。结论儿童持续脐带分泌物并伴有严重的皮肤擦伤,应对伴有胃粘膜异位的脐肠管囊肿进行诊断。
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Journal of Pediatric Surgery Case Reports
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