Histological differentiation between prenatally diagnosed choledochal cyst and type I cystic biliary atresia using liver biopsy specimens.

Because it is difficult to distinguish preoperatively between prenatally diagnosed choledochal cyst (CC) and type I cystic biliary atresia (BA) by ultrasound scanning or magnetic resonance imaging (MRI), some modality of discrimination for the 2 entities is required. The aim of this study was to investigate the histological differences between prenatally diagnosed CC and type I cystic BA using liver biopsy specimens. Four children with prenatally diagnosed CC and two children with prenatally diagnosed type I cystic BA were identified by fetal ultrasonography between 1985 and 2003. The control group included two children who were operated on at an earlier period due to postnatally diagnosed BA. Liver wedge biopsy in the right lobe was performed at the time of the radical operation. Histological findings of the H & E-stained liver biopsy specimens were classified into 4 grades (Grade 0, no abnormality; Grade 1, ductular proliferation without bridging fibrosis; Grade 2, ductular proliferation with bridging fibrosis; and Grade 3, liver cirrhosis). The CC group consisted of one case of Grade 0 and three cases of Grade 1. The type I cystic BA group consisted of one case each of Grade 2 and Grade 3. In the control group, both cases were Grade 2. The authors suggest that exploratory laparotomy might be avoided and, instead, histologic examination using liver biopsy specimens can be a reliable test for the differential diagnosis of CC and type I cystic BA in prenatally diagnosed neonates.     

超声检查在胆道闭锁鉴别诊断中的运用价值

目的 探讨超声检查胆囊大小、形态及肝门区结构在早期鉴别诊断胆道闭锁和婴儿肝炎综合征方面的运用价值。方法回顾2002～2006年204例拟诊为阻塞性黄疸的婴儿的超声检查资料，并与手术造影、临床诊断相比较，统计胆囊显示率、胆囊大小及其在胆道闭锁和婴儿肝炎综合征之间的差异；回顾2002～2006年的超声图像，综合分析胆囊充盈情况、形态及肝门区结构，评估其诊断价值。结果204例中，182例经手术探查及造影确诊，其中胆道闭锁（BA）151例（83％），婴儿肝炎综合征（IHS）29例（16％），先天性胆道发育不良2例（1％）。超声探及胆囊的BA患儿97例（占BA患儿的64.24％），探及胆囊的IHS患儿24例（古IHS患儿的82．76％），BA患儿胆囊显示率明显低于IHS患儿（P〈0．05）。BA患儿胆囊长度均值为1．28cm，体积均值为0．27ml；IHS患儿胆囊长度均值为2．03cm，体积均值为0．61ml，两组差异有统计学意义（P〈0．05）。回顾性分析胆囊、肝门区结构，诊断灵敏度为99．34％，特异度为83．87％，符合率为96．7％。结论超声检查综合分析胆囊大小、形态及肝门区结构在早期鉴别诊断胆道闭锁和婴儿肝炎综合征方面有一定的运用价值。     

Choledochal cysts in infancy and childhood

Objective  To study the clinical presentation, management, and results of treatment of 41 consecutive cases of choledochal cyst (CC) managed from 1999 to 2006. Methods  The age of the patients ranged from 20 days to 11 years. Eleven cases were 1 year or less in age (infantile group), and 30 patients were more than 1 year old (classical pediatric group). Children less than 1 year old presented with jaundice (72%), hepatomegaly (54%) and clay-colored stools (63%); whereas those above one year in age presented with pain (83.3%) and jaundice (47.6%). Three cases had biliary perforation with localized or generalized biliary peritonitis. Ultrasonography (US) diagnosed/suggested CC in all 41 patients, however, contrast-enhanced computed tomographic (CECT) scan (n=12), radionuclide DISIDA scan (n=5), and magnetic resonance cholangiopancreatogram (MRCP) (n=3) also contributed to the diagnosis. 19 patients, including 4 from the ‘infantile’ group, had significant dilatation of intrahepatic biliary ducts. Results  Nine patients from the ‘infantile’ group had cystic type I CC (type Ic), while 2 patients had fusiform dilatation of the common bile duct (type if disease). In contrast, 21 of the older patients had type If disease while 9 had type Ic disease. Operative management included primary complete excision of the cyst with a Roux-en-Y hepaticodochojejunostomy (HDJ) (n=32), Lilly’s modification of submucosal resection with a HDJ (n=8), and cyst excision with a hepaticodochoduodenostomy (HDD) (n=1). All 3 patients with biliary perforation had primary excision of the CC with a HDJ. Unusual operative findings included accessory hepatic ducts (n=2), anteriorly placed common or right hepatic arteries (n=3), and partially or completely blocked proximal extrahepatic bile ducts (n=3). There were 2 postoperative deaths, one had cirrhotic liver disease with portal hypertension, the other had deep icterus with poor general condition. Both patients succumbed to overwhelming sepsis. One patient required reexploration and refashioning of the HDJ for biliary leak. Short-term follow-up was satisfactory in all 39 patients. Conclusion  Infants with CC constitute a distinct group with regard to clinical presentation and the pathological anatomy. CC should be kept in mind while evaluating neonates and infants with cholestatic jaundice, and older children with recurrent abdominal pain. Primary excision of the cyst with a HDJ provides satisfactory results in the management of the vast majority of cases of CC including those in infants, massive CC, and in the presence of biliary perforation and peritonitis.     

Comparison between prenatally diagnosed choledochal cyst and type-1 cystic biliary atresia by CD56-immunostaining using liver biopsy specimens.

Because it is difficult to distinguish preoperatively between a prenatally diagnosed choledochal cyst (CC) and type-1 cystic biliary atresia (BA) by ultrasound scanning or magnetic resonance imaging, some mode of discriminating between the 2 entities is required. The aim of this study was to investigate the immunohistological differences between prenatally diagnosed CC and type-1 cystic BA, using liver biopsy specimens immunostained for CD56. Five children with prenatally diagnosed CC and two children with prenatally diagnosed type-1 cystic BA were identified by fetal ultrasonography between 1985 and 2004. The control group included two children who were operated on at an earlier period due to postnatally diagnosed BA. Liver wedge biopsy in the right lobe was performed at the time of the radical operation. Histological findings of the CD56-stained liver biopsy specimens were classified into 4 categories each, with particular focus on staining distribution and intensity. The staining distribution was classified according to the scale 0 = no staining; 1 = some staining of bile ducts/ductules but staining in less than one-third of portal tracts; 2 = staining in one-third to less than two-thirds of portal tracts; and 3 = staining in more than two-thirds of portal tracts. Staining intensity was scored as follows: 0 = no staining, 1 = weak staining, 2 = moderate staining, and 3 = strong staining. The staining intensity and distribution in the CC group was zero in all 5 cases. The type-1 cystic BA group consisted of patients with scale 1 or 3 staining distribution and score 1 or 2 staining intensity. In the control group, staining distribution was 1 or 3, and staining intensity was 1 or 3. These results indicate that CD56-positive biliary duct cells are present in prenatally diagnosed type-1 cystic BA. The authors suggest that exploratory laparotomy might be avoided and, instead, immunohistological examination using liver biopsy specimens may be a reliable test for the differential diagnosis of CC and type-1 cystic BA in prenatally diagnosed neonates.     

经腹腔镜手术治疗Ⅰ、Ⅱ型胆道闭锁

目的 对经腹腔镜手术治疗Ⅰ型及Ⅱ型胆道闭锁的效果进行探讨.方法 自2003年3月至2007年7月,共收治患Ⅰ型及Ⅱ型胆道闭锁并胆管囊性扩张的患儿10例,其中Ⅰ型8例,Ⅱ型2例;男4例,女6例;年龄23～160 d,平均53.8 d.患儿均有黄疸、陶土样便等症状;伴有总胆红素、转氨酶明显升高;术前经B超等影像学检查证实有肝门部囊性扩张的胆管存在,平均直径1.5 cm(1.0～1.8 cm);10例经术中证实为Ⅰ、Ⅱ型胆道闭锁伴有肝外胆道囊性扩张.本组10例患儿行经腹腔镜扩张的胆管底部切除胆管空肠Roux-en-Y吻合术.术后平均随访26个月(4～51个月).结果 本组10例患儿行经腹腔镜手术全部成功,无中转开腹.手术时间平均为3.0 h(2.4～3.2 h),术中出血量5～10ml.术后胃肠平均通气时间为18 h(16～28 h),进食时间平均20 h(16～30 h);平均术后3 d(2～4 d)排黄色大便;腹腔引流放置时间平均58 h(48～72 h),平均术后10 d(7～16 d)黄疸减轻,6例患儿术后14 d总胆红素直接胆红素降至正常水平,3例患儿3个月降至正常水平,1例手术年龄5.5个月患儿虽获良好胆汁引流,胆红素水平有所降低,但因肝硬化严重,后来仍维持高水平,胆汁引流后肝功能改善不佳,术后28 d死于肝功能衰竭.转氨酶术后不同程度的下降.术后平均住院时间6.8 d(5～9 d).术后随访4～51个月,1例患儿于术后4周发生胆管炎,经抗炎后痊愈.其余患儿肝功能各项指标均正常,无胆管炎、吻合口狭窄、粘连肠梗阻等术后并发症.结论 经腹腔镜囊性扩张的胆管底部切除胆管空肠吻合术治疗Ⅰ、Ⅱ型胆道闭锁是一种安全有效、可靠的方法.     

Comparison technetium of Tc-99m disofenin cholescintigraphy with ultrasonography in the differentiation of biliary atresia from other forms of neonatal jaundice

Technetium Tc-99m disofenin cholescintigraphy (CS) and ultrasonography (US) are two major clinical methods used in differentiating biliary atresia (BA) from neonatal jaundice. To compare the diagnostic utility of these two modalities, 66 patients with neonatal cholestasis (15 BA, 3 choledochal cyst (CC), 32 neonatal hepatitis, 13 prolonged jaundice, 2 total parenteral nutrition, and 1 sepsis) underwent Tc-99m disofenin CS and US. The diagnostic sensitivity, specificity, and accuracy of CS in differentiating BA from other forms of neonatal jaundice was 100%, 87.5%, and 90.5%, respectively, and for US 86.7%, 77.1%, and 79.4%, respectively. Tc-99m disofenin CS after premedication with phenobarbital and cholestyramine is a convenient and reliable method of differentiating BA from neonatal hepatitis, with a diagnostic accuracy superior to that of US. However, US is the initial imaging procedure of choice in patients presenting with jaundice to rule out anatomic anomalies such as CC.     

Correlation of Prenatal Ultrasound Diagnosis and Pathologic Findings in Fetal Anomalies

This retrospective study compared the prenatal ultrasound (US) diagnosis with autopsy findings in 61 intact fetuses following induced abortion and 36 fragmented fetuses from dilatation and evacuation (D&E). In intact fetuses, complete agreement between US diagnosis and autopsy findings was achieved in 65.6% of cases in the central nervous system (CNS) and 47.5% in other somatic organ systems (SOS). There were major differences between US and autopsy findings involving the CNS in 6.5% of cases and SOS in 27.9%. Correlation was better for evaluation of renal anomalies (complete agreement in 63.6% of 11 suspected cases, 2 false-positive and no false-negative cases) than congenital heart disease (complete agreement in 27.3% of 11 suspected cases, 5 false-positive and 3 false-negative cases). In D&E specimens, a prenatal diagnosis of neural tube defect (NTD) was confirmed in 90% of cases. However, due to fragmentation of fetal parts, the US diagnosis in the CNS could not be confirmed totally (69.4%) or partially (2.8%) in fetuses with chromosomal abnormalities (ChA) or multiple congenital anomalies (MCA). Nonetheless, the US diagnosis of SOS was confirmed in six cases on D&E, including Meckel-Gruber syndrome, cystic hygroma, renal agenesis with contralateral renal dysplasia, cardiac defect, fetal hydrops, and tracheal atresia. Our results show that a thorough autopsy of an intact fetus after abortion is necessary to confirm prenatal diagnosis and allow proper management and counseling. The pathologic examination of D&E specimens can reliably confirm the US diagnosis of NTD, but it is very limited in identifying other fetal anomalies. Received January 6, 1998; accepted May 25, 1998.     

产前诊断胆道闭锁影像学研究

目的：胆道闭锁早期诊断困难,而产前诊断更是极少发现。本文对产前诊断的胆道闭锁影像学特点进行探讨。方法回顾我院2010年至2012年收治的产前诊断9例产前诊断胆道畸形患儿,入院手术年龄24 d至2岁。全部患儿行腹腔镜胆道造影,4例诊断胆道闭锁,5例诊断先天性胆管扩张症,胆道闭锁患儿中2例接受腹腔镜下肝管空肠ROUX-Y吻合术,2例接受开腹肝门空肠吻合术。观察其临床表现,超声和实验室指标,术中情况,术后恢复情况等。结果4例患儿产前超声未见胆囊或胆囊显示不清。产前超声发现肝门部囊肿的3例患儿,囊肿小且均无明显增大,张力较高,呈规则圆形。产前诊断发现肝门囊肿的3例患儿术中证实为胆总管远端闭锁（Ⅰ型胆道闭锁）,未发现胆囊也未发现囊肿的1例患儿证实为Ⅲ型胆道闭锁。生后全部胆道闭锁患儿出现黄疸,最早出现在生后第2天,但都未出现陶土样便。全部胆道闭锁患儿囊肿大小形态无明显变化。全部患儿查ALT、AST、rGGT、直接胆红素和总胆红素进行性升高。2例接受腹腔镜下肝管空肠ROUX-Y吻合术,2例接受开腹肝门空肠吻合术。全部4例患儿术后恢复好。结论产前超声检查可以确定胆道闭锁,如果发现肝门部囊肿的胎儿,应定期接受超声检查,如果囊肿在孕期变化不明显,应怀疑囊肿型胆道闭锁。如产前超声未发现胆囊结构,则应怀疑为Ⅲ型胆道闭锁。生后应密切观察、超声、生化、黄疸情况。如果黄疸进行性加重可及早进行腹腔镜胆道造影及手术治疗。     

Pre-operative ultrasonographic diagnosis of biliary atresia—with reference to the presence or absence of the extrahepatic bile duct

Our aim was to evaluate the efficacy of ultrasonographic (US) examination in the pre-operative diagnosis of biliary atresia (BA) with special reference to the presence or absence of extrahepatic bile duct. Thirty consecutive neonates and infants aged 8 to 169 days (mean: 62 days) suspected of having biliary atresia were examined pre-operatively in real time B-mode ultrasonography. We used a 5 or 7.5 MHz probe of micro convex type. Patients were fasted and sedatives administered. When the common bile duct was absent, we considered it a positive finding for BA diagnosis; if not, it was considered a negative finding. A definitive diagnosis of BA was confirmed at surgery by gross morphology or intra-operative cholangiography. US findings had a sensitivity of 83% (19 of 23 BA patients), a specificity of 71% (5% of 7 non BA patients) and an accuracy rate of 80%. The positive predictive value was 90% (19 of 21), while the negative predictive value was 56% (5 of 9). There were four false-negative cases. Two were BA cases with patent distal common bile duct, one was BA in which the hepatic artery was determined to be the common bile duct, and the other was a subtype of extrahepatic bile duct dilatation (the so-called, "correctable type"). We employed US criteria for visualization of the extrahepatic bile duct for pre-operative diagnosis of BA. US examination referring to the presence or absence of the extrahepatic bile duct is an effective and useful method for clinical survey.     

Living-related liver transplantation for biliary atresia associated with polysplenia syndrome

This report describes a 1-yr-old boy with biliary atresia (BA) and polysplenia syndrome (PS) who underwent successful living-related liver transplantation (LTx). At the time of initial hepatic portoenterostomy, he was noticed to have a preduodenal portal vein (PV), non-rotation of the intestine, and polysplenia. Because he did not achieve good bile excretion, he underwent a living-related LTx (using a left lateral segment from his mother) at the age of 14 months. Evaluation of the vascular anatomy was made by angiography, magnetic resonance imaging (MRI), computerized tomography (CT), and Doppler ultrasound. The PV was stenotic from the confluence of the superior mesenteric vein (SMV) and splenic vein (SpV) to the hepatic hilum. The retrohepatic inferior vena cava (IVC) was deficient cranially to the renal vein and was connected to the azygous vein. The supra-hepatic IVC was detected below the diaphragm and was connected to three hepatic veins. The common hepatic artery (HA) originated from the superior mesenteric artery. At LTx, the PV was dissected to the level of confluence of the SMV and the SpV, from which the venous graft was interposed using the donor's ovarian vein. Three hepatic veins were plastied into one orifice, which was anastomosed to the graft's hepatic vein under the diaphragm. The graft vascularity and function has been good for 1 yr after LTx. In the present case, sufficient pre-LTx evaluation of vascular anomalies seemed to help performance of the successful LTx.     

胆道闭锁葛西手术后肝内胆管扩张的治疗与预后分析

目的 探讨胆道闭锁葛西手术后肝内胆管扩张（Intrahepatic biliary cysts,IBCs）的治疗选择和预后情况.方法 回顾性分析8例肝内胆管囊性扩张患儿的临床表现、治疗过程和转归,其中2例单发囊肿型胆管扩张患儿分别予手术和抗感染治疗;6例多发复杂囊肿型胆管扩张患儿均行经皮肝胆管引流术（PTCD）治疗.结果 2例单发囊肿型胆管扩张患儿疗效好,预后良好.6例多发复杂囊肿型胆管扩张患儿经PTCD引流后,2例再次开腹手术吻合;6例患儿中,3例死亡,1例行肝脏移植,1例等待肝移植,1例合并门静脉高压存活.结论 胆道闭锁葛西术后多发肝内胆管扩张（C型）常提示存在严重肝脏损伤,PTCD治疗或手术再吻合治疗多数情况下仅是姑息治疗.PTCD有助于减轻黄疸,改善肝移植术前条件;而无条件进行肝脏移植的患者,单纯PTCD很难长期控制临床症状,应选择再次手术行扩张胆管肠吻合手术.单发肝内胆管扩张（A型）临床过程和预后明显优于C型扩张,可能与肝脏受损程度较轻有关.     

Study of the Malformation of Ductal Plate of the Liver in Meckel Syndrome and Review of Other Syndromes Presenting with This Anomaly

Meckel syndrome (MIM 249.000) is an autosomal recessive disorder with a variable spectrum of anomalies. Since the first reports of this syndrome, very broad diagnostic criteria have been proposed, but the process of defining them continues. It is probable that at least two of three manifestations, including cystic kidney dysplasia, occipital encephalocele or other anomaly of the central nervous system, and postaxial polydactyly occur in most cases. Arrest of the development of intrahepatic bile ducts at the stage of the bilaminar plate formation or ductal plate malformation is considered of high diagnostic value in Meckel syndrome, but there is no complete agreement in the literature about its occurrence. The aims of this investigation were to study the prevalence and morphologic patterns of ductal plate malformation of the liver in Meckel syndrome by evaluating the dilatation of primitive biliary structures and the increase in connective tissue of the portal tract. Archival data files from four German centers (Berlin, Freiburg, Heidelberg, Mainz) were reviewed. Liver sections of 30 well-studied fetuses with Meckel syndrome were immunostained with antibodies against cytokeratins (intermediate filaments of the cytoskeleton) and factor VIII (an endothelial cell marker) and were evaluated both qualitatively and quantitatively. Cystic kidney dysplasia, occipital encephalocele, and postaxial polydactyly were found in 100%, 90%, and 83.3% of the fetuses, respectively. Ductal plate malformation of the liver was a constant anomaly in Meckel syndrome, seen as frequently as renal lesions. We observed essentially two kinds of hepatic lesions: 23 cases showed mainly a cystic dilatation of primitive biliary structures with little portal fibrosis, while 7 cases showed mainly rings of interrupted curved lumina around a central fibrovascular axis and pronounced portal fibrosis. In these seven cases an abnormal pattern of the portal vein, with many small and closely spaced branches of the portal vein (the so-called pollard willow pattern), was also seen. With respect to other fetal developmental anomalies, no difference between the two types of lesions was found. We also provide a potentially useful comprehensive review of other genetic syndromes in which ductal plate malformations may occur. Received May 14, 1999; accepted October 11, 1999.     

A case of biliary atresia with cystic dilatation of the extrahepatic bile duct and polysplenia syndrome

A 79-day-old girl presented with jaundice and acholia. Laboratory findings disclosed elevated levels of bilirubin, transaminases, and gamma-glutamyl transferase. A chest X-ray film showed dextrocardia. A computed tomographic scan revealed a cystic mass at the porta hepatis, multiple spleens in the right side of the abdomen, and absence of the inferior vena cava. Under the diagnosis of biliary atresia (BA) (I-cyst) associated with polysplenia syndrome, a hepatic portojejunostomy was performed at 80 days of age. The postoperative course was uneventful, and the jaundice cleared. Although the occurrence of associated anomalies in BA is rare, polysplenia is most commonly seen, and its incidence is reported to be 2% to 10%. Patients with BA and polysplenia usually have a poor prognosis, however, this patient may have a good prognosis due to uneventful recovery from the jaundice. Accepted: 30 August 1999     

Neonatal Adrenal Hemorrhage Detected Antenatally

This report describes two cases of neonatal adrenal mass detected antenatally by routine ultrasound (US) examination of pregnant women. Case 1 was recognized by the fetal US at 31 weeks gestation. The mass, located near the right upper pole of the kidney, was echolucent on US examination. A serial US of the mass showed changes of the internal echoes from a cystic lesion to a mixed lesion, and finally to a hyperechogenic lesion due to a neonatal adrenal hemorrhage (NAH). At 33 days, laparotomy was performed, and the pathological finding revealed an NAH owing to the mass bleeding into the adrenal cyst. Case 2 was also detected by fetal US just before birth. The mass of the right upper pole of the kidney was hyperechogenic on US examination. The baby clinically deteriorated after birth because of hypovolemia owing to NAH. A serial US of the mass showed the change from a hyperechogenic to a cystic lesion. Four months later, the mass spontaneously resolved. From the US spectrum, the mass was diagnosed as NAH. These are the second known cases of NAH detected before birth in Japan.     

Fetal ovarian cysts: postnatal evolution and indications for surgical treatment

Fetal ovarian cysts are an increasingly common finding during routine prenatal ultrasonography (US). These lesions were occasionally diagnosed in the past, but only on the basis of a palpable abdominal mass or, rarely, because a twisted ovarian cyst caused an acute abdomen. Surgery is currently recommended for neonates after prenatal diagnosis, even in asymptomatic cases, when the cyst has a diameter larger than 5 cm or echographic findings suggest torsion or intracystic hemorrhage. In our 12 cases of suspected fetal ovarian cysts, wide variations in diameter were seen in 6 during pre- and postnatal follow-up. Two of these were not confirmed at birth; 7 were treated conservatively and excluding 1 perinatal death from multiple associated anomalies and 1 case lost to follow-up, the remaining cases showed uneventful, spontaneous regression of the cyst. Postnatal US signs suggesting necrosis secondary to torsion in a previously uncomplicated cyst and requiring surgery were seen in only 3 cases. Conservative treatment must be recommended in most prenatally diagnosed cysts; indications for surgery cannot be based only on dimensions. The incidence of complications has been overemphasized and the risk of associated malignancy is negligible in newborns, while possible size reduction and resorption can be expected in most cases. Nonoperative management may also be recommended when signs of torsion are seen before birth and spontaneous asymptomatic involution of the cystic ovary is documented in early postnatal life. Accurate clinical and US follow-up is mandatory in all cases. Surgery must be reserved for symptomatic cases or when an alteration of the cystic structure is documented by US in a previously uncomplicated cyst.     

Oxidative stress profile in the post-operative patients with biliary atresia

Background and purpose  Many post-operative patients with biliary atresia (BA) suffer from liver dysfunction, such as chronic inflammation even without jaundice after a Kasai’s hepatic portoenterostomy. Methods  The presence and degree of oxidative stress were evaluated in the post-operative patients with BA. Twelve outpatients who underwent a Kasai’s hepatic portoenterostomy were evaluated. The active oxygen products, the rate of bioantioxidant, the markers of oxidative stress, and the degree of hepatic oxidative stress were examined by immunohistochemical staining of biopsied specimens. Results  All of the oxidative stress markers in the post-operative patients with BA increased in comparison to those in the controls. Moreover, 8-OHdG immunohistochemical staining was positive in 84 ± 4.8% in hepatic cells in the portal area in the post-operative patients with BA. Conclusion  The post-operative patients with BA were under increased oxidative stress, even if their liver dysfunction was mild without jaundice. Antioxidant therapy might be necessary to decrease of oxidative stress in the post-operative patients with BA.     

不同年龄先天性胆管扩张症的临床观察

目的：探讨不同年龄先天性胆管扩张症的特征，病因及防治措施。方法：对1988年1月－1998年12月收治的213例先天性胆管扩张症按年龄分3组，将分型，临床表现，治疗及预后等相关因素综合分析。结果：（1）新生儿、婴儿17例，囊肿型16例（94．12％），临床以黄疸（11例）、的（6例）为主要症状，15例手术治疗，13例（86．67％）治愈，2例（13．33％）死亡。（2）幼儿，儿童组163例，均以间歇性腹痛就诊，囊肿型74例（45．40％），梭型89型（54．60％），76例行影像学检查，62例（81．58％）伴胰胆管合流异常。161例手术治疗，治愈160例（99．38％），死亡1例（0．62％）。（3）成人组33例，囊肿型及混合型占多数，因反复感染并发症日益增多，33例中24例（72．73％）合并胆管结石等并发症，癌变2例，手术治疗33例，治愈30例（90．91％），死亡3例（9．09％）。结论：本病应根据不同年龄，临床表现，采用现代的影像技术检查，做到早期诊断、早期行根治术。     

Postnatal management of cystic neuroblastoma.

Cystic adrenal neuroblastoma (NB) is highly unusual. We report two cases of cystic NB, detected antenatally and emphasize postnatal strategy management. Case 1: a right cystic mass was detected in a foetus in the 34th week of pregnancy, and checked as the same in the 36th week. Postnatal ultrasonography confirmed the presence of a growing adrenal cystic mass. MRI and MIBG scintigraphy suggested an isolated adrenal tumour, without catecholamine secretion. Surgical resection was decided upon and pathological examination confirmed the diagnosis of cystic NB. Follow-up found a recurrence at 10 months and the patient underwent excision after chemotherapy. Histology confirmed the metastatic origin from NB. Case 2: a right adrenal cystic mass was detected during pregnancy, with no regression of tumour size on postnatal US. MRI, MIBG scintigraphy, and catechol plasma and urinalysis led to the conclusion of an isolated non-secreting lesion. A right adrenalectomy was performed at 1 month. Pathologic examination diagnosed a cystic NB. We would suggest serial US examinations and early removal of any cystic mass with characteristic US signs (thick complex wall) or without regression on one month follow-up. Prenatal diagnosis may provide the best chances for neonatal excision and good prognosis.     

MR imaging of fetal cerebral anomalies

Background. Prenatal diagnosis of fetal brain anomalies relies mainly upon ultrasonography. However, even in the most experienced hands, the technique has limitations for some difficult diagnoses. MRI is an excellent imaging modality for the paediatric and adult brain. Objective. To assess the value of prenatal MRI when a cerebral anomaly was detected by US and where the prognosis depended on the identification of other anomalies undetectable by US, or where fetuses were at risk for a CNS lesion even when the US was normal. Materials and methods. Four hundred prenatal MRI examinations were performed since 1988, and confirmed by postnatal follow-up or pathological examination. Two-thirds of the examinations were performed after 25 weeks of gestation, one-third between 21 and 26 weeks. Fetal immobilisation was obtained by maternal premedication with flunitrazepam, administered orally 1 h before the examination. The examinations were performed on 1.5 T scanners using one or two surface coils. Results. Prenatal MRI allowed the diagnosis of serious unsuspected lesions such as neuronal migration disorders, ischaemic and haemorrhagic lesions and the abnormalities observed in tuberous sclerosis. It helped to characterise ventricular dilatation and anomalies of the corpus callosum and of the posterior fossa. Conclusions. MRI is a valuable complementary tool when prenatal US is incomplete, doubtful or limited. Prenatal MRI is particularly useful for the detection of ischaemic and haemorrhagic lesions, neuronal migration disorders and tuberous sclerosis lesions. Detection of these associated anomalies worsens the fetal prognosis, has medico-legal implications and modifies obstetric management. Normal prenatal MRI does not exclude an anomaly. Received: 3 November 1997 Accepted: 7 November 1997     

胆道闭锁术后肝内胆管囊性扩张的诊治

目的总结胆道闭锁术后肝内胆管扩张的诊治经验，探讨葛西手术后肝内胆管扩张对患儿远期预后的影响，以及如何早期诊断和治疗。方法2003年4月至2008年3月，对3例因胆道闭锁行葛西手术的患儿进行追踪随访，3例术后均有不同程度胆管炎症状，其中1例合并门脉高压。3例行超声、CT或经皮肝穿刺置管引流（PTCD），结果显示肝内胆管囊性扩张。2例行胆管扩张与空肠胆支再吻合术，1例仅行PTCD置管引流。结果2例经手术治疗的患儿，术后黄疸消退或减轻。1例仅行PTCD的患儿肝内胆管扩张长期存在。结论肝内胆管扩张使胆管炎反复发作，特别是扩张的囊状胆管压迫门静脉，可使门静脉变窄，血流减少，致受累肝叶萎缩。胆道闭锁患儿葛西手术后应定期行超声检查，及时发现肝内胆管囊性扩张。葛西手术后肝内胆管扩张应早期诊断，早期手术治疗，术前应常规行PTCD，暂时解除胆汁淤积，并为术中定位做好准备。