儿童先天性门体分流的诊治北大核心CSCD

目的总结儿童先天性门体分流的诊治经验。方法收集2014年1月至2021年4月收治于上海交通大学医学院附属上海儿童医学中心普外科及心内科的20例先天性门体分流患儿临床资料,其中男15例,女5例。肝外门体分流9例,肝内门体分流9例,肝外合并肝内门体分流2例。4例患儿合并先天性心脏病,2例合并肝脏良性肿瘤。治疗方法采用血管内介入、手术结扎、介入与手术结合以及肝移植。分析患儿围手术期及术后随访的临床数据。结果介入封堵分流以及手术结扎共14例,肝移植手术6例。9例肝外门体分流中,门腔分流6例,门-左肾静脉分流2例,肠系膜下静脉与髂内静脉分流1例。其中,6例一次手术结扎,2例延迟分次结扎,1例肝移植。9例肝内门体分流中,单纯封堵3例,手术结扎2例,手术加封堵1例,肝移植3例。2例肝外合并肝内门体分流均行肝移植。所有患儿术前症状均得到改善,1例肝移植术后出现脑出血。随访2~82个月,没有死亡病例,患儿生长发育较好。结论儿童先天性门体分流应按个体化方案进行治疗,血管介入治疗为首选方式,手术结扎也是重要手段,特殊病例最终需要肝移植。     

先天性肝外门腔静脉分流并下消化道出血1例

先天性肝外门腔静脉分流是一种罕见的先天性门静脉畸形,Abernethy在1793年首次报道,也称为Abernethy畸形[1]。国内外文献报道60余例[2.3.4.5]。而先天性肝外门腔静脉分流并发下消化道出血的病例,文献     

并发消化道出血的先天性门体分流畸形8例影像学特征病例系列报告

摘要目的：探讨合并消化道出血的先天性门体分流畸形的影像学特征,以提高对该畸形的认识。方法：纳入复旦大学附属儿科医院病历系统中先天性门体分流且病史中有消化道出血症状的病例,采集性别、年龄、临床表现、CTA和MRA影像学资料、外科手术结局。CTA为GE Light speed 64排螺旋CT扫描仪,MRA为Siemens Avanto 1.5 T 扫描仪,提取纳入病例的CTA原始图像,以1.25 mm层厚/0.625 mm层间隔重建,传入后处理工作站进行多平面重建;提取纳入病例的MRA原始图像,传入MR工作站进行后期多平面重建;由2名从事儿科影像诊断10年以上医生独立读片,观察门静脉及其属支的形态及走行,异常门体分流位置、途径,髂静脉、下腔静脉及肠管、肠壁血管分布。结果：2008年3月至2017年1月符合本文病例纳入标准的连续病例8例,男6例,年龄3月至10岁,7例贫血,7例便血,1例呕血。8例均行CTA检查,其中2例同时行MRA检查。截止出院时,6例行手术治疗,2例随访观察。8例先天性门体分流畸形患儿均为肝外型门体分流,2例肝外型Ⅰ型,6例为Ⅱ型。以便血为表现的7例中,髂内静脉、直肠上静脉及结肠静脉扩张扭曲;以呕血为表现的1例,脾静脉和肠系膜上静脉汇合后通过胃冠状静脉汇入左肾静脉,胃冠状静脉显著扩张、扭曲。结论：合并便血的先天性门体分流大部分通过肠系膜下静脉与髂静脉异常沟通,发生便血的比例高。     

改良Soave术与Sarasola-Klose内痔环切术联合治疗儿童肛门、直结肠弥漫性血管畸形

目的 介绍改良Soave术结合Sarasola-Klose内痔环切术联合治疗儿童肛门、直肠、结肠血管畸形.方法 回顾分析2000年至2008年收治的12例小儿肛门、直肠、乙状结肠血管畸形患儿的临床资料,就其诊断和手术治疗进行分析.结果 根据病史、纤维结肠镜检查、钡剂灌肠、腹部CT和MRI检查,临床诊断为弥漫性肛门、直肠、乙状结肠血管畸形.11例患儿接受改良经腹Soave术,肛周黏膜层及黏膜下血管切除采用Sarasola-Klose内痔环切术式(改良Whitehead术).1例患儿仅接受常规经腹Soave术.12例患儿术后随访3个月～8年,1例仍有少量间歇性便血,余均无便血、便秘,1例污粪.结论 血管畸形累及肛门、直肠甚至乙状结肠的病例少见,肠镜、CT、MRI是诊断的主要方法.改良Soave术结合Sarasola-Klose内痔环切术是安全、彻底、有效的治疗方法.     

儿童Abernethy畸形1例报告并文献复习

Abernethy畸形又称先天性肝外门腔静脉分流畸形,其主要的特征为门静脉部分或全部缺失,原门静脉血未经肝脏直接汇入腔静脉,从而使肝功能异常及胃肠道静脉血运障碍。该病于1793年由John Abernethy在女婴尸检中首次发现,于1997年命名为Abernethy畸形[1]。该畸形十分罕见,全世界仅有百余例报道,临床医生对于该畸形认识不够深入,容易误诊。现将宁夏医科大学总医院小儿外科收治的1例Abernethy畸形患儿报告如下。     

儿童先天性肺动静脉畸形临床诊治分析

目的探讨儿童先天性肺动静脉畸形的临床特点,评估CT血管成像(CTA)和数字减影血管造影(DSA术在其诊断中的价值。方法回顾性分析2015年4月至2020年5月南京医科大学附属儿童医院收治的7例先天性肺动静脉畸形患儿的临床特征、实验室检查、影像学资料及治疗转归。结果 (1)肺动静脉瘘4例:以紫绀及杵状指为主要表现,CTA均提示肺动静脉畸形。1例手术切除右下肺;2例于胸腔镜下对累及肺动静脉瘘结扎;1例通过DSA对右下动静脉瘘处封堵栓塞。1例尚住院治疗,3例随访1～5年紫绀及杵状指明显改善。(2)支气管动脉-肺动脉瘘3例,均以咯血为主要表现。2例行CTA检查,其中1例提示右肺支气管动脉增粗迂曲,1例未发现异常支气管动脉;3例均行DSA术,术中均见异常支气管动脉分支显影及分流,2例予以支气管动脉封堵栓塞治疗,其中1例封堵2个月后又出现咯血,再次行封堵,随访3年无咯血,另有1例因分流较小,予临床观察,随访15个月无咯血。结论不明原因咯血、紫绀、杵状指的患儿需考虑先天性肺动静脉畸形,CTA或DSA检查有利于明确诊断,DSA检查较CTA更能直观地观察到受累血管及其走行,并能对受累的血管范围及其复杂程度进行判断。     

先天性胆总管囊肿诊治52例报道

目的探讨先天性胆总管囊肿的诊断与治疗方法。方法对我院2002年1月～2007年1月收治的52例先天性胆总管囊肿患儿进行回顾性分析。结果52例均经B超、CT、MRCP等检查诊断为先天性胆总管囊肿,并行手术治疗,手术方式为胆总管囊肿切除、肝门空肠Roux-Y型吻合术。全组无死亡病例,无重大并发症,预后良好。结论B超是诊断先天性胆总管囊肿的首选方法,胆总管囊肿切除、肝门空肠Roux-Y型吻合术是治疗先天性胆总管囊肿的首选方法。     

应用B超诊断婴幼儿结直肠息肉

目的 探讨应用B超诊断结直肠息肉的价值。方法 对因便血怀疑结肠息肉的婴幼儿73例，均先进行B超检查看能否发现结肠息肉，随后均行电子结肠镜检查，以证实结直肠息肉的存在。结果 患儿73例行B超检查发现结直肠息肉患儿37例，经电子结肠镜检查后发现73例便血患儿39例存在息肉，B超发现37例均被证实为结直肠息肉。结论 B超是诊断婴幼儿结直肠息肉的一种简便、无创的方法，可应用于结直肠息肉的筛查。     

移植血管间置、门静脉主干-门静脉肝内左支分流术在治疗小儿肝外门静脉高压中的应用

目的 探讨移植血管间置、门静脉主干-肝内门静脉左支分流术在治疗小儿肝外门静脉高压中的效果和预后.方法 回顾性分析2010年1月至2014年12月9例(男6例,女3例)因“呕血、黑便”等上消化道出血表现入院,入院后完善腹部CT、超声及门静脉血管B型超声检查,诊断为门静脉海绵样变、门静脉高压和脾大患儿的临床资料.所有患儿均行移植血管间置、门静脉主干-肝内门静脉左支分流术治疗,术中造影发现胃冠状静脉直径较细或长度不够,不能行胃冠状静脉-门静脉左支分流术治疗,遂采用移植自体血管的方式,将其两端分别与门静脉主干和肝内门静脉左支吻合.其中采用移植空肠静脉方法治疗2例,回肠静脉4例,肠系膜下静脉3例.患儿术后均获随访,随访时间1～60个月,平均24.6个月.随访期间,采用超声评估脾大小及分流血管通畅情况.记录血常规、血生化和血氨,评估脾功能亢进情况和肝功能.结果 所有患儿均成功行移植血管间置、门静脉主干-门静脉肝内左支分流术治疗.手术时间105～360 min,平均218.3 min.其中3例住院期间有输血记录.术后住院时间6～10d,平均7.2d.术后复查B型超声示脾长径为9.6～14.7 cm,厚径为3.4～5.4 cm;较术前(长径10.4～17.6 cm,厚径3.2～6.8 cm)明显降低.血常规、生化和血氨均恢复正常.随访B型超声可见分流血管通畅,血管直径为0.5～0.7 cm,平均0.6 cm;分流血管入肝血流速度为0.14～0.18 m/s,平均0.16 m/s.1例患儿复发,采用保守治疗有效.结论 移植血管间置、门静脉主干-门静脉肝内左支分流术是治疗小儿肝外门静脉高压的有效方式之一,在患儿不能采用胃冠状静脉-门静脉左支分流术治疗时可考虑采用该方法治疗.     

先天性门体静脉分流相关性肺动脉高压5例临床分析

目的探讨儿童先天性门体静脉分流相关性肺动脉高压(PAH-CPS)的临床特点。方法回顾分析2012年1月至2019年1月收治的PAH-CPS患儿的临床资料。结果共5例患儿,男2例、女3例,初诊年龄0.08～4.4岁。主要表现为反复肺部感染、肝脾大、肝功能异常、肺动脉高压。超声心动图检查明确患儿均有不同程度肺动脉高压、合并心内畸形。腹部超声示肝内外门体静脉分流。腹部增强CT和心导管造影可显示肝内外血管的走形和发育。结论 PAH-CPS可表现为反复肺部感染、肝脾肿大、肝功能异常等;超声检查可提供诊断线索,腹部增强CT扫描、心导管造影可明确诊断。     

Embolization of congenital portosystemic shunt presenting after pediatric liver transplantation: Case report and literature review

This case report describes a 13‐year 10‐month‐old girl who underwent a deceased‐donor split LT for primary diagnosis of biliary atresia at the age of 12 months, who presented with a lower GI bleed. Ultrasound and CT revealed a venous vascular anomaly involving the cecum and ascending colon, with communication of the SMV and pelvic veins consistent with a CEPS. Associated varices were noted in the pelvis along the uterus and urinary bladder. These findings were confirmed by trans‐hepatic porto‐venography, which was diagnostic and therapeutic as a successful embolization of the CEPS was performed using micro‐coils. There were no complications following the procedure and no further GI bleeding occurred, illustrating the efficacy of this treatment option for CEPS. We discuss the literature regarding the presenting complaint of GI bleeding post–LT, CEPS as a rare cause of GI bleeding and its association with PV, and the classification and treatment of CEPS.     

Portal obstruction in children. I. Clinical investigation and hemorrhage risk

We examined 108 children with obstruction of the portal vein. Symptoms included splenomegaly and gastrointestinal tract hemorrhage. Obstruction was secondary to portal vein injury in 44 children and was combined with congenital malformations in 17 others. Ultrasonography provided the correct diagnosis in 36 of the 37 children in whom it was performed. Angiography, performed in 101 children, showed that the obstruction extended to the superior mesenteric vein in 14 children and to the entire portal venous system in seven; intrahepatic branches were involved in half the cases. Natural splenorenal shunts were visible in 19 children but were not clearly associated with a lower risk of gastrointestinal tract bleeding; in five of 30 children, cavography displayed abnormalities of the inferior vena cava. Spontaneous gastrointestinal tract hemorrhage occurred in 78 children. Fiberoptic endoscopy showed esophageal varices in 79 of the 81 children studied. The presence of tense varices and congestion of esophageal mucosa clearly augmented the risk of bleeding. These results suggest a simple method of investigation based on ultrasonography for diagnosis and on endoscopy for prognosis. Angiography should be limited to children with a history of gastrointestinal tract bleeding for whom a surgical portosystemic shunt is being considered.     

Congenital extrahepatic portosystemic shunt associated with heterotaxy and polysplenia

Background

Heterotaxy with polysplenia is associated with many cardiovascular anomalies including the occasional occurrence of congenital extrahepatic portosystemic shunts (CEPS). Missing this anomaly can lead to inappropriate and ineffective therapy.

Objective

To emphasize the importance and associated anatomy of CEPS in conjunction with heterotaxy with polysplenia.

Materials and methods

Review of three young children who presented with cyanosis and pulmonary hypertension without a cardiac etiology. They were known (1) or discovered (2) to have heterotaxy with polysplenia.

Results

There was absence of the intrahepatic inferior vena cava (IVC) with azygos or hemiazygos continuation in all three cases. In spite of normal liver function, they were discovered to have large portosystemic shunts, splenorenal in location, along with diffuse peripheral pulmonary arterial dilatation suggestive of CEPS (Abernethy malformation) with hepatopulmonary or, more accurately, portopulmonary syndrome. All CEPS were ipsilateral to the spleens. Patency of the portal veins in these cases allowed for percutaneous shunt closure with resolution of cyanosis.

Conclusion

CEPS is associated with heterotaxy with polysplenia and can be symptomatic because of pulmonary arteriovenous (AV) shunting. Portal and hepatic vein patency are critical for determining feasibility of CEPS closure.     

Sigmoidoscopy, colonoscopy, and radiology in the evaluation of children with rectal bleeding

The authors evaluated the diagnostic role of sigmoidoscopy, colonoscopy, and double contrast radiology in 103 children with rectal bleeding, with or without other gastrointestinal symptoms. The children's mean age was 44 months, with a range from 1 month to 12 years. In 74.5% of the subjects investigated, visual inspection of the anus and sigmoidoscopy with rectal biopsy disclosed a positive diagnosis. Of the remaining patients, a conclusive diagnosis was reached by either colonoscopy or double contrast radiology in all but six patients. These six, with mild painless hematochezia, remained without a diagnosis. The diagnostic procedure in pediatric patients with rectal bleeding should include an initial visual inspection of the anus, and sigmoidoscopy; air contrast enema and colonoscopy should be performed only in children whose sigmoidoscopy is negative, in diagnostic assessment of inflammatory bowel disease, and in cases of recurrent bleeding after removal of rectal polyps. Colonoscopy is important also in the follow-up examination of children with inflammatory bowel disease and allows the removal of polyps located in the proximal colon.     

Portosystemic shunting in children during the era of endoscopic therapy: improved postoperative growth parameters

BACKGROUND: Surgical portosystemic shunting has been performed less frequently in recent years. In this retrospective study, recent outcomes of portosystemic shunting in children are described, to evaluate its role in the era of endoscopic therapy. METHODS: Retrospective chart review of children who underwent surgical portosystemic shunt procedures between October 1994 and October 1997. RESULTS: Twelve children (age range, 1-16 years) underwent shunting procedures. The causes of portal hypertension were extrahepatic portal vein thrombosis (n = 6), congenital hepatic fibrosis (n = 2), hepatic cirrhosis (n = 2), and other (n = 2). None of the patients were immediate candidates for liver transplantation. Types of shunt included: distal splenorenal (n = 10), portocaval (n = 1), and other (n = 1). Median follow-up was 35 months (range, 24-48 months). All patients are currently alive and well with patent shunts. The mean hospital stay was 8 days. Three patients required readmission for further interventions because of shunt stenosis in two and small bowel obstruction in the other. Mild portosystemic encephalopathy was seen in one child with pre-existing neurobehavioral disturbance. Excluding a patient who underwent placement of a portosystemic shunt for a complication of liver transplantation, mean weight-for-age z score in nine prepubertal patients improved from -1.16 SD to +0.15 SD (P = 0.023), and mean height-for-age z score from -1.23 SD to 0.00 SD (P = 0.048) by 2 years after surgery. CONCLUSIONS: Surgical portosystemic shunting is a safe and effective method for the management of portal hypertension in childhood. Patients show significant improvements in growth parameters after the procedure. Surgical portosystemic shunting should be actively considered in selected children with portal hypertension.     

Surgical intervention in children with portal hypertension

Over a 5-year period 252 children with bleeding oesophageal varices secondary to portal hypertension were treated by endoscopic injection scletherapy. Surgical treatment was restricted to patients with bleeding complications following sclerotherapy and the occasional problem of symptomatic splenomegaly (2 children). Sclerotherapy failure was due to the development of varices not accessible to sclerotherapy (gastric fundus 7, duodenum 1, jejunum 1, unidentified site in small bowel 1) and to continued bleeding from oesophageal ulcers in 2 children. There were 5 children with Child's grade A cirrhosis. Fifteen children (4.4 % of the total; median age 8.7 years, range 1.5 – 18 years) underwent 16 operations during this period, which included 13 porto-systemic shunts (8 lieno-renal and 5 meso-caval) and 3 oesophageal transections/devascularisations. Sclerotherapy was not attempted in 1 of the 2 children treated for continued bleeding from oesophageal varices. Two lieno-renal shunts thrombosed and 1 of these patients underwent a meso-caval shunt. There were no deaths in this group of patients during a median follow-up of 40 months. One child with biliary atresia has developed a mild intermittent encephalopathy. The use of injection sclerotherapy for bleeding oesophageal varices with surgical intervention limited to patients failing treatment resulted in low morbidity and no mortality in a large group of children with portal hypertension. Correspondence to: N. D. Heaton     

Transcatheter Diagnosis and Intervention for Iatrogenic Right-to-Left Shunts Decades After Surgical Repair of Partial Anomalous Pulmonary Veins and an Atrial Septal Defect

A 57-year-old man presented with symptomatic right-to-left shunts decades after surgical closure of an atrial septal defect and partial anomalous pulmonary veins. Two sources of shunting were identified: a baffle leak from the right superior vena cava (RSVC) to the left atrium secondary to complete occlusion of the RSVC-to-right atrium connection and an acquired previously unpublished coronary sinus to the left atrial shunt secondary to inadvertent closure of the coronary sinus. The diagnosis and transcatheter management of these unusual right-to-left shunts are reported.     

Trans-anal protrusion of ventriculo-peritoneal shunt catheter with silent bowel perforation: report of ten cases in children

Ventriculo-peritoneal (VP) shunting used in the treatment for hydrocephalus is associated with several complications. Mechanical failure of shunt is the commonest complication of all. Visceral/bowel perforation is an unusual but serious complication of VP shunting. This article reports our experience in the management of ten children who had VP Shunt catheter protrusion from anus. This is a retrospective study of ten patients who had VP shunt catheter protrusion from anus, admitted in the department of paediatric surgery between Jan 1996 and Dec 2005. The records of above ten cases were reviewed for their clinical presentation and management, etc. We had performed 398 VP shunt operations in the last 10 years. Two hundred and seventy one (68.09%) VP Shunts were done for congenital hydrocephalus of which 164 were done in infancy/neonatal period and 107 VP shunts were done in the age group of >1–12 years. One hundred and twenty-seven (31.90%) VP shunt operations were done for patients who had hydrocephalus as a complication following tubercular meningitis (TBM). Out of 398 VP shunts, ten patients (2.51%) had protrusion of the distal end of peritoneal catheter from anus without causing/leading to peritonitis. We observed a 08.29% mortality of all VP shunt operations. Protrusion of VP shunt catheter per rectum can occur without producing peritonitis. Formal exploration and localization of entry of VP shunt catheter in bowel is not mandatory. Mini laparotomy and revision of peritoneal part of shunt can be done if there is no shunt infection.     

A single-pass tunneling technique for CSF shunting procedures

Implantation of ventriculoperitoneal shunts in the precoronal position is generally accomplished using a retroauricular incision for subcutaneous tunneling. Retroauricular incisions can be associated with complications, including cerebrospinal fluid leak and shunt infection. We describe a technique for 'single-pass' shunt tunneling from frontal to abdominal incisions and our initial results in a consecutive, prospective series of 15 children (age 2 days to 5 years). Eleven patients presented with congenital hydrocephalus (including 5 with myelomeningocele and 3 with posthemorrhagic hydrocephalus) and 4 with hydrocephalus secondary to central nervous system (CNS) tumors. The average length of clinical follow-up was 6 months (range 1-13 months). There were no perioperative or long-term complications of the single-pass technique. Nine of the 11 patients with congenital hydrocephalus are currently well without any further medical or surgical intervention. Two underwent shunt revision for proximal obstruction, with an intact distal system. Three of the 4 patients with hydrocephalus secondary to CNS tumor suffered secondary shunt complications during periods of severe neutropenia resulting from chemotherapy (6 weeks to 6 months after shunt insertion). For primary ventriculoperitoneal shunt insertion in infants and young children, the single-pass tunneling technique is safe and avoids one source of complications.