Biliary atresia is an obliterative disorder of the bile ducts, causing obstructive jaundice in neonates. In this study, the developing biliary system of normal human embryos and fetuses was examined and compared with the resected extrahepatic biliary remnants from 205 cases of biliary atresia. At the porta hepatis level, it was found that the primary biliary ductal plate undergoes a specific sequence of remodelling, resulting in the formation of large tubular bile ducts surrounded by thick mesenchyme, between 11 and 13 weeks postfertilisation. These developing ducts are in luminal continuity with the extrahepatic biliary tree throughout gestation. Contrary to long-held belief, no "solid phase" was observed in the development of the extrahepatic bile duct. Examination of the biliary remnants in biliary atresia showed that the porta hepatis is encased in fibrous tissue, and a variable pattern of obliteration of the common hepatic and common bile ducts was observed. Anticytokeratin immunostaining showed similarities between the abnormal ductules within the porta hepatis in biliary atresia, and the developing bile ducts in the first trimester. Biliary atresia may be caused by failure of the remodelling process at the hepatic hilum, with persistence of fetal bile ducts poorly supported by mesenchyme. As bile flow increases perinatally, bile leakage from these abnormal ducts may trigger an intense inflammatory reaction, with subsequent obliteration of the biliary tree.

Resected extrahepatic remnants taken at the time of portoenterostomy were examined in a single-center review of 205 cases of biliary atresia. The morphological features of the size and number of residual ducts at the porta hepatis and the degree of inflammation at the porta hepatis were analyzed using a semiquantitative scoring system. The morphology of the common hepatic and common bile duct was classified into seven types. These features were then related to age at time of initial surgery and to survival. This showed that few or absent ductal remnants at the porta hepatis and absence of portal inflammation were predictors of poor prognosis. These histological features may represent the “burnt out” end result of the disease process. There was no correlation between age at time of portoenterostomy and either portal duct patency or portal inflammation. The common hepatic and common bile duct were variably involved in the sclerosing process, but the patterns of obliteration were not indicative of prognosis. The severity of intrahepatic biliary cholangiopathy and the extent of liver damage may ultimately be more important to survival in the long term.

A 14 year old girl with multiseptate gall bladder and cystic dilatation of the biliary tree is presented. This is the 20th published case report of patients with multiseptate gall bladder and only the second to be associated with a choledochal cyst. The cystic spaces of the gall bladder did not communicate with the neck of the gall bladder or the rest of the biliary tree, and this unusual feature has not been previously described. A multiseptate gall bladder with a normal biliary tree commonly causes symptoms suggestive of cholecystitis, although gall stones are seldom present. Diagnosis is confirmed by an oral cholecystogram or ultrasound scan that may show the fine intraluminal septae, and these features should be looked for in patients with biliary symptoms without biliary calculi. Cholecystectomy is curative for the isolated gall bladder anomaly but hepaticojejunostomy may be necessary for an associated choledochal cyst.

A 10-year-old boy presented with a 5-month history of recurrent abdominal pain, fever, and jaundice. Ultrasound examination revealed a thick-walled gallbladder and dilatation of the biliary tree. Endoscopic retrograde cholangiography showed irregular common bile duct dilatation. After cholecystectomy for acalculous cholecystitis, histological examination of the operative specimen revealed the features of xanthogranulomatous cholecystitis. The abnormalities observed in the bile duct were secondary. This unusual destructive inflammatory process has previously only been described in adults.

Histological features in liver biopsy specimens taken from 71 infants at the time of surgery for biliary atresia (portoenterostomy) were analyzed using a scoring system and compared with an endoscopic grading of esophageal varices performed at a mean age of 3.4 years. The analysis showed no correlation between a "global" score, which represented the severity of all histological changes in the original biopsy specimen, and the severity of esophageal varices. Further analysis also showed no correlation with any individual histological feature (eg, fibrosis). These findings failed to confirm a previous study, which suggested a relationship between liver changes at surgery and the later development of esophageal varices in children with biliary atresia.

Normal development of the human lower urinary tract was studied between the 14th and 20th week of gestation using 3 modes of fixation. Fixation by direct distension provides a high degree of reproducibility of parameters used to study the growth of the fetal bladder. Using this method, fetuses ranging from 12 to 21 weeks gestation were studied. Results obtained demonstrate that the length of the bladder, the inter-ureteric distance, and the distance between the apex of the trigone and the distal tip of the urethra occur in a linear mode. Furthermore, the rate of growth of the male urethra was evidently higher when compared to that of the female from the 12th week of gestation. Data from this work can be used for a more accurate assessment of cases with abnormal lower urinary tract development.

Fifty placentas were collected after vaginal delivery or cesarean section from normal and abnormal pregnancies and were fixed under different conditions of perfusion using a peristaltic roller pump. In each case, a physiologic-heparin perfusate was used for less than 10 minutes, followed by a buffered solution of glutaraldehyde-formaldehyde. The best results were obtained with placentas from cesarean sections perfused immediately after delivery with a pressure maintained under 60 mm Hg. Placentas of this group were fixed within 30 minutes and electron microscopy demonstrated good preservation of cellular ultrastructure. Perfusion fixation could be performed up to 6 hours after delivery with satisfactory histologic results. In these cases, electron microscopy revealed ischemic changes 10 minutes after delivery and severe necrosis 1 hour after delivery. When the perfusion pressure was maintained over 60 mm Hg, diffuse damage of the villous morphology was observed. Histomorphometric analysis showed significant differences between terminal villi from nonperfused (immersed-fixed) placentas and perfused-fixed placentas. The mean barrier and trophoblastic thicknesses and the mean volume fraction of trophoblast were significantly (P < .001) increased in the nonperfused group compared with the perfused group.

Journal of Ultrasound in MedicineVolume 9, Issue 7 p. 419-422 Case Reports An angiomyxoma involving the whole length of the umbilical cord. Prenatal diagnosis by ultrasonography. E Jauniaux, E Jauniaux Department of Obstetrics and Gynecology, King's College Hospital, King's College School of Medicine and Dentistry, Denmark.Search for more papers by this authorG Moscoso, G Moscoso Department of Obstetrics and Gynecology, King's College Hospital, King's College School of Medicine and Dentistry, Denmark.Search for more papers by this authorL Chitty, L Chitty Department of Obstetrics and Gynecology, King's College Hospital, King's College School of Medicine and Dentistry, Denmark.Search for more papers by this authorD Gibb, D Gibb Department of Obstetrics and Gynecology, King's College Hospital, King's College School of Medicine and Dentistry, Denmark.Search for more papers by this authorM Driver, M Driver Department of Obstetrics and Gynecology, King's College Hospital, King's College School of Medicine and Dentistry, Denmark.Search for more papers by this authorS Campbell, S Campbell Department of Obstetrics and Gynecology, King's College Hospital, King's College School of Medicine and Dentistry, Denmark.Search for more papers by this author E Jauniaux, E Jauniaux Department of Obstetrics and Gynecology, King's College Hospital, King's College School of Medicine and Dentistry, Denmark.Search for more papers by this authorG Moscoso, G Moscoso Department of Obstetrics and Gynecology, King's College Hospital, King's College School of Medicine and Dentistry, Denmark.Search for more papers by this authorL Chitty, L Chitty Department of Obstetrics and Gynecology, King's College Hospital, King's College School of Medicine and Dentistry, Denmark.Search for more papers by this authorD Gibb, D Gibb Department of Obstetrics and Gynecology, King's College Hospital, King's College School of Medicine and Dentistry, Denmark.Search for more papers by this authorM Driver, M Driver Department of Obstetrics and Gynecology, King's College Hospital, King's College School of Medicine and Dentistry, Denmark.Search for more papers by this authorS Campbell, S Campbell Department of Obstetrics and Gynecology, King's College Hospital, King's College School of Medicine and Dentistry, Denmark.Search for more papers by this author First published: 01 July 1990 https://doi.org/10.7863/jum.1990.9.7.419Citations: 26AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume9, Issue7Jul 1990Pages 419-422 RelatedInformation

Twenty pregnancies with elevated maternal serum alpha-fetoprotein (MSAFP), a normal fetus and unusual or abnormal placental/cord sonographic features are reported. These include: (A) gigantic enlargement with multiple sonolucent spaces of different size and shape (n = 2; Swiss cheese); (B) placental masses of variable echogenicity (n = 5); (C) cord masses with central echo-dense zone and peripheral hypoechoic areas (n = 2); (D) enlarged placentas with patchy decrease of echogenicity (n = 6; jelly-like); and (E) large sonolucent spaces with turbulent blood flow surrounded by normal placental tissue (n = 5; placental lakes). After delivery, these ultrasound features were compared with pathologic findings. Diffuse mesenchymal hyperplasia of the stem villi were found in the gigantic placentas (n = 2). The placental masses corresponded to chorioangiomas (n = 3), infarct (n = 1) or subamniotic hematoma (n = 1) and the cord masses to angiomyxomas (n = 2). The 'jelly-like' placentas were related to subchorial thrombosis (n = 2), massive fibrin deposition (n = 1) or hypertrophy with no obvious abnormalities (n = 3). Large subchorial thrombosis (n = 2), or no obvious abnormalities (n = 3) were observed in placentas with large lakes. These findings suggest that a large range of placental and cord anomalies are associated with elevated MSAFP and are potentially diagnosable by routine sonographic examination at the time of AFP screening.

Ultrasonographic features of a fetus at 18 weeks of gestation suggesting a body stalk anomaly are presented. These included a large abdominal anterior wall defect in apparent continuity with the placenta, severe kyphoscoliosis of the lower spine, the absence of one kidney, and a very short umbilical cord with only one umbilical artery. The amniotic fluid was reduced and the fetus was almost immobile at short‐interval ultrasound examinations. The pregnancy was terminated and autopsy of the fetus showed abnormalities compatible with maldevelopment of both cephalic and caudal embryonic folds.

Ciliogenesis of the respiratory epithelium in the human cartilaginous trachea start during the 12th week of gestation. Ciliary shafts are first seen under the scanning electron microscope during the 13th week. Unlike its membranous counterpart, ciliary shafts appear all over the epithelial surface at almost the same time. Epithelial cells destined to become ciliated cells first develop numerous long and thin microvilli. A process of individual cell extrusion and proliferation of neuroepithelial bodies around the carinal angle precede ciliation in the respiratory epithelium of the cartilaginous trachea. Epithelial cell differentiation patterns in both the cartilaginous and membranous trachea are different. The mechanisms involved in modulating cell differentiation are currently under investigation.

Segmental imaging studies of the respiratory epithelium from human embryos and fetuses of normal karyotype have demonstrated that ciliogenesis and ciliation of the respiratory epithelium starts at 7 weeks of gestation. Ciliated cell differentiation follows a pre-determined pattern of distribution. It starts exclusively in the upper segment of the membranous trachea and spreads distally. Ciliation of the carinal angle takes place at 8 weeks of gestation. Three patterns of basal body formation were identified. The various morphological features encountered are described and compared with those observed in cases of Immotile Cilia Syndrome and other pathological conditions. Ciliation of the respiratory epithelium in the cartilaginous trachea does not take place until after the 12th week of gestation. The morphological findings identified in our case material are in agreement with those observed in the developing respiratory epithelium of other higher mammals.

A study into the normal anatomy of the venous circulation of the gastroesophageal junction was undertaken using three complementary techniques (radiology, corrosion casting, and morphometry). Four distinct zones of venous drainage were defined as follows: (a) gastric zone, characterized by a longitudinal venous distribution; (b) palisade zone, composed of parallel vessels arranged in groups, lying mainly within the lamina propria; (c) perforating zone, characterized by "treble clef" shaped veins, which collect and channel blood into extrinsic veins; and (d) truncal zone, composed of four or five deep lying descending veins. This venous system appeared to be mainly distributed within the esophageal mucosal folds. The anatomic pattern suggests that venous flow is bidirectional at the palisade zone, which acts as a high-resistance watershed region between the portal and azygos systems. In patients with portal hypertension this normal vascular system has to accommodate greatly increased venous flow, and the anatomy as demonstrated here offers insight into variceal development.

With the increasing use of ultrasound examination in the antenatal period the lack of morphological correlates has become a problem. This study was carried out in order to correct this deficiency. An antenatal real-time ultrasound scan of the fetal head was performed and measurements of the biparietal diameter, occipitofrontal diameter, head circumference and cerebral ventricle-to-hemisphere ratio were undertaken in 103 fetuses at 13-24 weeks gestation. After prostaglandin termination of pregnancy, the fetal brains were perfused in situ through the right carotid artery with 10% formal saline. Horizontal slices of the fetal head were cut, photographs were obtained and measurements of the same parameters as in the ultrasound examination were undertaken. There is a significant correlation (p less than 0.001) between the ultrasonographic and post-mortem measurements of all parameters. Also several anatomical structures of the fetal brain are now confidently identified in the antenatal ultrasound scan, using the anatomical preparations for comparison.

Pathological changes identified in the muscular wall of the stomach from two young insulin-dependent diabetic patients suffering from severe gastric autonomic neuropathy are presented. Scattered smooth muscle cells appearing as homogeneous round eosino philic bodies (“M” bodies) among areas of sub-total smooth muscle cell atrophy together with intercellular collagen proliferation were identified in the muscularis pro prig of the stomach. Ultrastructurally, the “M” bodies are transformed smooth muscle cells undergoing a form of necrobiosis with peculiar intracellular features. These changes appear characteristic of end-stage diabetic gastric autonomic neuropathy when compared with other pathological conditions involving the gastric wall. The degree and extent of involvement of the various components of the gastric wall are discussed.