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Home , Cystic vein, Ductus venosus, Systemic venous system, Venous plexus

Annals of the Royal College of Surgeons of England (I974) vol 55

Surgical implications of portal venous system malformation

Charles Marks MD MS PhD FRCP FRCS FACS Professor of Surgery, Louisiana State University School of Medicine; Surgeon, Charity Hospital in New Orleans

Summary where they empty into the primitive sinus The significance of congenital abnormalities venosus via hepatocardiac channels (Fig. i). in predisposing to portal hypertension and A venous plexus in the caudal portion of variceal haemorrhage needs to be remem- bered when these effects manifest in child- RIGHT hood, as portal venography will permit BUD elucidation of the complicated congenital RT. POSTERIOR LT; ANTERIOR developmental abnormalities underlying the CARDINAL CARDINAL VEIN pathological condition and permit rational surgical amelioration. In the presence of portal hypertension the development of a collateral venous circula- ...\t l--l~~~~~~~~~~...... tion may be represented by a hepatopetal ~~~~~~~.,:~~ ...... CARDINAL ~~~~~~~~~~~~~~...".iN-...... 1 ...... or hepatofugal circulatory pattern and will SINUS closely parallel the developmental areas where portal and systemic venous circulations meet, being representative of the embryological ANASTOMOSVI ...... anastomosis between the vitelloumbilical sys- tem and the posterior cardinal system of .

RIGHT Embryological development of the VEIN IN. portal vein The vitelline and umbilical veins provide VITELLINE venous drainage from the primitive intestinal LEFT tract and lead, in complex fashion, to the hA | iX VITELLINE VEIN development of the portal venous system. VI TELGINEDUCT | |GUT Vitelline veins The right and left vitel- line veins develop within the splanchnopleural FIG. I Diagrammatic illustration of relation- mesoderm of the wall and extend ship of vitelline veins to midgut in 6-week to the caudal edge of the septum transversum, . 300 Charles Marks the yolk sac wall communicates with thc An S-shaped portal vein is formed and as- umbilical veins via the vitelloumbilical anasto- sumes its adult form because of the plexiform mosis. nature of the embryonic veins and the effect In the 5-week embryo communicating ve- of haemodynamic forces applied along the nous anastomoses develop between the right venous pathway. As the stomach and duo- and left vitelline veins to form a figure-of- denum elongate and rotate from their eiglht pattern in relation to that part of the embryonic midsagittal to adult position the foregut destined to become the duodenum. venous blood proceeds from the left to the The primitive anastomoses include: (i) a right vitelline vein via the dorsal communica- ventral anastomosis caudal to the duodenal tion (Fig. 2). entry of the common bile duct anlage; (2) a Atrophy of the vitelloumbilical duct causes dorsal retroduodenal anastomosis caudal to the distal portion of the vitelline veins to the anlage of the dorsal pancreas; and (3) a atrophy as haemodynamic forces bypass these ventral communication in the region of the vessels. As the superior mesenteric vein differ- porta hepatis. entiates within the mesentery it drains into the left vitelline vein. The splenic vein joins Normal adult configuration Persistence the left vitelline vein at a higher level and of the normal adult portal vein configuration receives the inferior mesenteric vein. depends upon obliteration of: (i) the cranial component of the left vitelline vein; (2) the Umbilical veins The right umbilical caudal component of the right vitelline vein; vein disappears in the 6-7-mm embryo and (3) the ventral intervitelline anastomosis. after undergoing atrophy, so that all the blood from the enters the embryo through the left into the hepa- tic sinusoids. With the elaboration of the right side of the an enlargement of the hepatic sinusoidal com- munication occurs between the point of entry of the left umbilical vein and the right hepato- cardiac channel, which is the persistent prox- imal part of the right vitelline vein. This channel provides the ductus venosus. After the left umbilical vein and the ductus venosus are obliterated and are represented in postnatal life by the liga- mentum teres and the ligamentum venostum respectively. Congenital abnormalities of the portal venous system 9mm normal 9 mm pre-du:cInol Preduodenal and prebiliary configura- FIG. 2 Mechanisms in normal and preduo- tion ofthe portal vein The foramen of denal development of portal vein in 9-mm Winslow or opening to the lesser sac has fetus. at its anterior margin the right free border Surgical implications of portal venous system malformation 301 of the hepatoduodenal . Within this it was joined by two mesenteric veins. The structure are enclosed the common bile duct, portal vein thus formed ran anterior to the the hepatic artery, and the portal vein. The superior duodenal loop and into the lesser normal and conventional anatomical arrange- omentum anterolateral to the common bile rment has the hepatic artery to the left of duct and hepatic artery. the common bile duct and the portal vein Persistence of both the caudal and cepha- situated posterior and between these two struc- lic embryological loops will account for the tures. This arrangement is significant because portal vein anomaly described by Fraser and of the frequent need to explore the common Kingsley-Brown' in which a small normally bile duct in patients with gallstones and com- situated portal vein was associated with a mon duct disorders and when it is necessary large anterior portal channel with multiple to mobilize the portal vein as a preliminary varicosities. to procedures. The portal vein may enter directly into This arrangement may occasionally be al- the inferior vena cava without traversing the tered so that the portal vein lies anterior to or it may enter directly into the the common bile duct and hepatic artery. The right . infrequency of a congenital preduodenal por- Absence of the vena cava, though a rare tal vein has recently been emphasized by condition, has been described by Bernard7, in which case its tributaries drain into the Johnson1, and in over i,ooo operative pro- cedures on the portal triad carried out in portal vein. Hypoplasia of the portal vein may occur, the past I o years only 3 patients with a prebiliary portal vein have been found. The with compensatory varicose enlargement of rarity of this condition has also been re- the inferior mesenteric vein and marked ferred to by Greatrex2, Boles and Smith3, hypertension in that segment of the portal and Stengel4. venous system. Under these abnormal circumstances the Duplication of the portal vein Re- arrangement is altered so that the portal vein duplication of the portal vein is an uncom- lies anterior to the common bile duct and mon, albeit a real, developmental anomaly hepatic artery. This rare anomaly is rep- of the portal venous system. A second pre- resented by the portal vein occupying an duodenal portal vein may develop as a result anterior position in the free edge of the lesser of the confluence of the superior mesenteric omentum. It crosses the anterior aspect of vein and the inferior mesenteric vein, which the common bile duct and common hepatic traverses the transverse mesocolon to reach duct, and the persistent caudal-ventral com- the ventral surface of the third part of the municating channel which provides the pre- duodenum and then runs anteriorly to the duodenal component can cause duodenal head of the pancreas. Reaching the gastro- obstruction. hepatic ligament, it then courses anterior to Knight5 recorded an anomalous portal vein the bile duct and hepatic artery to reach the which constituted a surgical danger by vir- liver to the left of the fossa, where tue of the splenic vein crossing posterior to it penetrates the quadrate lobe of that organ. the mid-pancreas and emerging just below the The normally situated portal vein is found in pancreatic neck before passing along the sum- such a case as a continuation of the splenic mit of the duodenojejunal flexure to come to vein and is situated in its customary retro- lie anterior to the head of the pancreas, where duodenal retropancreatic situation from where 302 Charles Marks it reaches the hepatoduodenal ligament to corded in I9I3 in association with multiple course to the porta hepatis in its customary cardiac defects12 and was described 3 years relationship to the common bile duct and the later as an isolated entity'3. Weinberg and hepatic artery. Kolson'4 described a triad consisting of pul- Such a malformation may give rise to por- monary-portal venous communication, mul- tal hypertension, with the development of tiple intracardiac anomalies, and partial situs oesophagogastric varices, and may provide a inversus. source of fatal haemorrhage during childhood. During the period I952-72 I,782 patients In such a case described by Snavely and with congenital disease have been Breakell8 two separate portal veins were pre- studied at Charity Hospital in New Orleans. sent and these passed over the anterior sur- In this group there were i8 patients with face of the second and third parts of the TAPVD, providing an incidence of i % of all duodenum and the head of the pancreas to cardiac malformations diagnosed clinically. In be joined together in a preduodenal anasto- this group of i 8 patients there were 3 with motic loop at the level of the lower border infradiaphragmatic drainage of the pulmon- of the duodenum. The significance of these congenital abnor- malities in predisposing to portal hypertension and variceal haemorrhage needs to be re- membered, and portal venography would be required to elucidate the congenital developmental abnormality underlying the pathological condition. Portal venous communication with the pulmonary venous system Anomalous pulmonary venous drainage to the portal vein represents a rare expression of total anoma- lous pulmonary venous drainage (TAPVD). TAPVD implies connection of the pulmonary venous system with the systemic system in- stead of the left atrium. Although it may exist alone, at which time it is potentially curable, it is more frequently found in asso- ciation with other complex cardiac anomalies. TAPVD was first described in 1798 in as- sociation with other major cardiac anomalies9 and in i868 as an isolated lesion'0. The in- frequency of the condition was noted by Maude Abbott1", who found only 4 cases in i,ooo consecutive autopsies of congenital heart disease. Only i of these 4 was found FIG. 3 Cardiac catheterization with angio- as a solitary lesion. graphy demonstrates transposition of pul- The rare variety of subdiaphragmatic monary veins with drainage infradiaphragm- drainage of the pulmonary veins was first re- atically into portal vein. Surgical implications of portal venous system malformation 303 ary veins and in only one of this latter excessive in extent, then involvement of the group was the pulmonary-portal venous com- portal vein may provide a source for portal munication an isolated entity without other vein atresia or stenosis, with resulting por- associated cardiac defects (Fig. 3). tal vein obstruction, splenomegaly, variceal Anomalous pulmonary venous drainage of haemorrhage, and all the components of por- the infracardiac type invariably results in ob- tal hypertension, or Banti's syndrome as this struction to the pulmonary venous return and condition is also called. accordingly the young patients present early Neonatal omphalitis with extension of and deteriorate rapidly, death being inevitable thrombosis along the umbilical vein to the unless surgical alleviation can be successfully portal vein has also been invoked as a further accomplished. The infrequency of successful possible cause of portal vein obstruction in surgical correction is reflected in the fact the very young infant, with the subsequent that to date there are only 7 examples15. development of collateral circulation and The very fact, however, that surgical suc- gastrointestinal variceal haemorrhage. cess is possible emphasizes the significance Congenital portal vein atresia may involve of an understanding of the developmental the whole extent of the vein or may be local- aberration, its clinical features, and its man- ized to the portion just proximal to its division agement. into two main branches in the porta hepa- tis (Fig. 4). The embryological considerations Portal vein atresia The fetal umbilical previously considered readily account for this vein and ductus venosus, which empty into site predilection (Fig. 5). the left portal vein, normally undergo a spon- The clinical problems resulting from such taneous obliterative process within the first 3 a congenital abnormality, with the risk of weeks after birth, as previously mentioned. If serious and recurrent exsanguinating variceal this obliterative process should prove to be haemorrhage, require a portal decompression

FIG. 4 Diagrammatic rep- Z ., W enp resentation of portal vein ,rmaved atresia. R.SaAtro- Po.ncrealaco 304 Charles Marks Kobrichl" and Klemperer" have described in detail the anatomical peculiarities of this condition, but for a long time there has been controversy regarding the underlying patho- genesis of cavernous or cavernomatous trans- formation of the portal vein. There are three prevailing theories regard- ing the pathogenesis of this condition: i) Angiomatous malformation of the portal vein. Pick18 was a strong proponent of the view that the condition is a neoplastic or angiomatous malformation of the portal vein. 2) Sequel to portal vein thrombosis. The general consensus of opinion considers this condition to be due to portal vein throm- bosis with subsequent recanalization and compensatory enlargement of the collateral and . 3) Combination theory. Beitzke" and Hart20 suggested that fundamental failure of portal vein development accounted for the ex- ...... tensive cavernous malformation, but Meyer2' and Cabred"2 attempted to combine both these theories and suggested that the caver- nous formation represents an angiomatous FIG. 5 Splenoportography demonstrates site tumour superimposed on a congenital mal- of portal vein atresia. formation. Contemporary studies complemented by in the absence of a vein lumen and portal splenoportographic evaluation of such cases with a vein that is very small in such splenic would indicate that the original conceptions young infants. The creation of an anastomosis of Klemperer are the correct ones and strong- between the end of the divided inferior vena ly suggest that the formation of this cavernous cava and the side of the superior mesenteric venous lake results from recanalization of a vein a mesocaval connection which provides portal vein thrombosis which may well have will serve to reduce the venous pres- portal been superimposed on phlebitis of the vein sure and thereby prevent serious hamorrhage compounded by the opening up of collateral (Fig. 6). venules within the hepatoduodenal ligament Cavernous transformation of the and the portal vein area, providing the classic portal vein spongy venous lake-like appearance. This rare condition represents a replacement Strongly favouring this concept is the fact of the portal vein by a spongy, trabeculated that most of these patients do not present venous lake involving the area of the portal with clinical sequelae attributable to portal vein and extending into the gastroduodenal hypertension until the 2nd or 3rd decades, ligament. whereas if this were a truly congenital con- Surgical implications of portal venous system malformation 305

FIG. 6 Successful mesen- terocaval anastomosis de- monstrated by splenoporto- graphy relieves effects of portal vein atresia.

dition many more of these cases would pre- patterns: sent at an earlier age. i) Hepatopetal circulation With portal vein obstruction at any intrahepatic level Cruveilhier -- Baumgarten syndrome The development of prominent subcutaneous blood is shunted through the accessory veins collateral veins is a well-known compensatory of Sappey, which pass through the follow- manifestation of portal hypertension. Equally ing: (a) the peritoneal covering of the liver; classical is the periumbilical caput medusae (b) from the stomach into the stem of the appearance of these vesels. On occasion, portal vein or into the substance of the liver through persistence of a paraumbilical vein of via the ; (c) the epiploic veins Burrow or recanalization of the umbilical of the gastrohepatic omentum; (d) the hepato- vein, a single subcutaneous channel becomes colic and hepatorenal veins; (e) the dia- grossly varicose, exhibiting a venous 'hum' phragmatic veins; and (f) veins of the sus- and providing indication that there is obstruc- pensory ligament of the liver. tion to the portal flow at a site proximal to 2) Hepatofugal circulation This is asso- the entry of this vein, so that generally this ciated with intrahepatic causes of portal will represent intrahepatic cirrhotic disease. hypertension in which blood is shunted from This varicose abdominal subcutaneous vein the and spleen around is referred to as the Cruveilhier-Baumgarten the liver. The hepatofugal flow is closely syndrome and is important only as a rep- related to embryological venous development. resentative example of a varicose state of an (a) The veins located at the sites of the gastro- embryologically residual vessel. intestinal tract where columnar epithelium meets squamous epithelium-that is, at the Embryological basis of collateral cardia and anus; the former provides an out- circulation in portal obstruction let to the via the With obstruction of the portal vein the col- oesophageal veins of the azygos system, the lateral venous circulation develops along two latter provides an outlet via the middle 306 Charles Marks haemorrhoidal vein. (b) The veins at the site 9 Wilson, J A (I 798) Philosophical Transactions of the obliterated -that is, of the Royal Society of London. Series B. Bio- the periumbilical veins in the round ligament logical Sciences, 88, 346. of the liver. (c) The veins at sites within the io Friedlowshy, A (i868) Vierteljahrschrift fur die where the gastrointestinal tract and praktische Heilkunde, 25, 45. its appendages or derived structures become ii Abbott, M (0936) Atlas of Congenital Heart retroperitoneal developmentally or where they Disease, New York, American Heart Association. adhere to the abdominal wall as a result of I2 Rivet, L, and Girard, L (1913) Archives med- pathological adhesion formation, thereby pro- icales du Coeur, 6, 720. viding the retroperitoneal veins of Retzius. I3 Gohn, A (I9I6) Beitrdge zur pathologischen An- atomie und zur allgemeinen Pathologie, 62, I75. References 14 Weinberg, T, and Kolson, J W (I949) Bulletin I Johnson, G F (197I) American Journal of of the International Association of Medical Roentgenology, Radium Therapy, and Nuclear Museums, 30, 68. Medicine, I12, 93. I5 Marks, C, anid Albert, H (I974) Journal of 2 Greatrex, H G (I965) British Journal of Surgery, Caraiovascular Surgery, I5, 405. 52, 477. I6 Kobrich, R (I903) Inaugural Dissertation, Uni- 3 Boles, E T, and Smith, B (I96I) Pediatrics, versity of Kiel. 28, 805. I7 Klemperer, P (I928) Archives of Pathology, 6, 4 Stengel, F (I924) Zeitschrift fiur Anatomie und 353. Entwicklungsgeschichte, I02, 19I. i8 Pick, L (I909) Virchows Archiv fur pathologische 5 Knight, H D (I92I) Annals of Surgery, 74, 697. Anatomie und Physiologie und fur klinische Medizin, I97, 490. 6 Fraser, J, and Brown, A K (I944) Surgery, Gyne- cology and Obstetrics, 78, 520. I9 Beitzke, H (I910) Charite'-Annalen, 34, 446. 7 Bernard, C (I950) Archives ge'ne'rales de med- 20 Hart, C (1913) Berliner klinische Wochenschrift, ecine, 23, 36O. 50, 223. 21 F G Medizinische 8 Snavely, J G, and Breakell, E S (I954) American Meyer, (I920) Klinik, i6, 876. Journal of Medicine, i 6, 459. 22 Cabred, R G (I926) Semana me'dica, 33, 109.