The causes of neonatal jaundice are numerous and often obstructive in type. The surgeon should be involved at an early stage in the diagnosis: if surgery is indicated the earlier it is undertaken the better. The causes of jaundice may be divided into four main groups: anatomical, of which extrahepatic biliary atresia is the most common; metabolic, of which &agr;&sub1;-antitrypsin deficiency is the most frequent; infections which are usually viral; and miscellaneous, including intravenous alimentation and haemolytic disease.

A programme of investigations should be pursued vigorously and preferably be completed by 6 weeks of age, to permit early surgical intervention. Ultrasound and HIDA scans should be undertaken early in the course of the investigations, since their results often indicate the need for other tests. Extrahepatic biliary atresia, spontaneous perforation of the common bile duct, inspissated bile syndrome, and choledochal cyst are the surgical causes of neonatal jaundice.

Liver disease during early life has a deleterious effect on physical growth and maturation and adversely affects intellectual development. As patient survival rates improve careful detailed monitoring of patients' nutritional status is imperative, and aggressive nutritional support should be offered to such children including candidates for liver transplantation.

This is the most common surgical hepatobiliary disorder of new born infants, and the most common indicator for liver transplantation in childhood. The incidence is one per 14000 live caucasian births, and is 10 times higher in Polynesians and Hawaiians.

Part or all of the extrahepatic and intrahepatic biliary tree is affected by a sclerotic obliterative process which commences in the late prenatal period or perinatal period. There are three broad groups of patients with this condition, classified by the level at which the biliary tree is affected (Fig. 1) 2152: type I, atresia of the common bile duct with patent proximal ducts; type II, atresia of the hepatic duct with patent gallbladder and common bile duct; and type III, atresia of all extrahepatic ducts. Unfortunately the majority of infants (85–90 per cent) fall into type II or III, and are termed ‘uncorrectable’. Patency of the proximal ducts enables a drainage procedure to be performed in type I disease, which is therefore called the ‘correctable form’ (10–15 per cent).

Possible aetiological factors which have been suggested include ischaemic insult, Epstein-Barr or rubella virus infection, an autoimmune cause/phenomenon, disorders of innervation, and damage to the ducts by pancreatic enzymes or various toxins. None of these suggested causes has been confirmed, nor has any genetic factor been identified. Although the majority of patients suffer from isolated biliary atresia, 20 per cent have associated cardiovascular and portal anomalies, and it has also been reported as part of the polysplenia syndrome, and in association with &agr;&sub1;antitrypsin deficiency, and Duchenne's muscular dystrophy.

The periductal tissues of the biliary tree show marked inflammatory cell infiltrate, with degeneration of the biliary epithelial cells and obliteration of the lumen. The inflammatory process ascends progressively and results in oedema, widening of the portal tracts with marked cellular infiltrate, and bile duct reduplication. The main intralobular bile ducts are progressively destroyed over a period of several weeks. Electron microscopy shows degrees of luminal obstruction of the intrahepatic ducts, extending from bile ductules to main hepatic ducts, even in the ‘correctable’ type of biliary atresia. The inflammatory response gives way to marked increase in fibrous tissue around the portal tracts, extending into and replacing much of the normal hepatic parenchyma. Giant cells are not usually present. Fibrosis and cirrhosis eventually supervene, unless adequate, preferably early, surgical drainage is established.

The infants present within the first few weeks of life with a history of fluctuating but progressive jaundice, dark urine, and pale stools. Normal meconium is passed initially and the stools may be bile-coloured for a short period afterwards, but acholic stools are usual. The babies initially thrive. The liver may be palpable and soft, but becomes firm and enlarged with time. Splenomegaly and ascites are not usually present early in the disease. The fluctuating nature of the icterus and the history in some infants of the passage, in the early weeks, of a normally coloured stool, may cause considerable delay in referring the baby for investigation. Antenatal ultrasound may cause prenatal suspicion of the diagnosis, which can be confirmed early in the postnatal period.

Examination of a fresh stool is important: the presence of bile pigment in the stools makes the diagnosis highly unlikely. Ultrasound and HIDA scan should be performed, the former after a 4-h fast to produce optimal views of the gallbladder. The single most helpful (not necessarily diagnostic) investigation is the HIDA liver scintiscan, which often strongly suggests a parenchymal (i.e. medical) or obstructive (surgical) cause for the jaundice. Hepatitis or intrahepatic causes of cholestasis can be more clearly distinguished from extrahepatic biliary atresia if phenobarbitone is administered prior to the scan for approximately 3 days.

Despite the careful use of all modern investigative techniques, it still may not be possible to distinguish extrahepatic biliary atresia from neonatal hepatitis in an obstructive phase. Liver biopsy, taken in conjunction with HIDA scintiscan and examined by an experienced histopathologist, is usually, but not always, diagnostic in this regard. Clinical examination should have excluded the other important diagnostic pitfall, Alagille's syndrome (see below), while the liver biopsy should suggest the non-syndromic form of biliary hypoplasia.

When a diagnosis of extrahepatic biliary atresia is made, the correct treatment is early surgical exploration with a view to establishing biliary drainage, usually by the technique of portoenterostomy (or one of its modification) as described by Kasai in 1959.

Vitamin K and prophylactic antibiotics should be given pre-operatively. The structures in the free edge of the lesser omentum are approached and identified through a generous upper abdominal subcostal incision. If the preoperative diagnosis is correct, the extrahepatic biliary ducts will soon be seen to be absent, or represented by tiny threads. A small gallbladder is usually found, and peroperative cholangiography through the gallbladder may allow the biliary anatomy to be established. In the authors' experience, this is frequently unhelpful in deciding how to proceed. The use of modern technology in preoperative investigations means that it should be quite exceptional for surgical exploration to be commenced for a patient who did not have extrahepatic biliary atresia. If this were to occur the operating surgeon should have sufficient experience to recognize the anatomical normality of the ducts, without the aid of cholangiography. This view regarding the use of peroperative cholangiography would not, however, be shared by all surgeons.

Portoenterostomy is performed by taking down the gallbladder remnant and the cystic duct and dissecting all the tissues from the surface of the hepatic arteries and portal vein, right up to the porta hepatis above the T bifurcation of the portal vein. The V-shaped central fibrous core which contains the remnants of the bile duct is dissected up into the substance of the liver and down behind the bifurcation of left and right portal veins which is retracted downwards. The core is then transected. Some surgeons believe that the optimum level of transection of the core can be determined by frozen section with identification of true bile duct remnants, as opposed to ‘biliary glands or collecting ductules’. The desirability or feasibility of this degree of histological precision is not universally accepted.

Following transection of the core, a Roux limb is fashioned using upper jejunum: a length exceeding 40 cm is believed to reduce the incidence of subsequent cholangitis. An anastomosis is made using absorbable interrupted sutures, placing the back row first, then advancing the Roux limb to the porta and tying the sutures. The anterior wall is then completed in a similar manner. Patients in whom successful bile drainage is established frequently suffer recurrent cholangitis, which is a serious adverse prognostic event: numerous modifications of Kasai's original technique have been used to try to reduce the incidence of this complication. Venting the Roux loop by establishing a skin stoma has now been abandoned as this failed to reduce the incidence of cholangitis and was itself associated with morbidity. Antireflux procedures based on the ileocaecal valve; using the mobilized appendix as a conduit or intussuscepting the Roux loop on itself, are being evaluated. Repeat surgery for patients in whom initial bile drainage fades in the early postoperative period is also controversial, and its incidence is falling as transplantation becomes more successful.

Postoperatively, the appearance of bile in the stool and a slow but gradual decrease in enzymes and serum bilirubin levels is indicative of success. Even when bile does appear, its flow may not be sustained, or it may be insufficient to render the infant anicteric or to prevent ongoing liver fibrosis and deterioration of function.

The outlook depends on several inter-related factors, including the age of the patient at operation, the degree of fibrosis present on initial liver biopsy, the diameter and number of biliary ductules in the excised fibrous core, the volume and concentration of the bile drainage, and the avoidance of cholangitis with its deleterious effect on liver function. Age at operation is the most important of these. Survival with good biliary drainage is seen in 62.5 per cent of patients after 3 years when surgery was undertaken before 30 days of age. This compares with a survival rate at 3 years of only 28.8 per cent in babies undergoing surgery after the age of 120 days. Survival figures vary greatly and while this is in part due to the experience and expertise in a given centre, it is not the only explanation. Just as there are racial differences in incidence, there are indications that caucasian patients do less well than non-white races, even after adjusting for other prognostic factors.

Optimum results are seen in centres whose surgeons are experienced in this condition, operating on a minimum of five or more cases per year. Without corrective surgery, most patients die before 18 months of age. Of those undergoing surgery, approximately 50 per cent (20–60 per cent worldwide) will achieve sustained good bile drainage and normal growth. A further 25 per cent survive with icterus, portal hypertension, and/or some degree of liver function impairment, fibrosis, and cirrhosis. Approximately 50 per cent of all long-term survivors develop portal hypertension. Twenty-five per cent die early in the course of the disease despite a portoenterostomy. Some Japanese centres have reported that 75 per cent survive to live normal lives, largely due to early referral, 4-week postnatal check (not 6 weeks), a high index of suspicion, and meticulous surgery. Corrective surgery before the age of 60 days can achieve normal serum bilirubin in 80 per cent of patients, and at 3 years of age 40 per cent are free of jaundice.

When surgical drainage fails, the patient becomes a candidate for liver transplantation, even if the infant weighs as little as 2.5 kg. The indications for transplant include severe pruritus, failure to thrive, recurrent cholangitis, and deteriorating hepatic synthetic function. The health of candidates for transplantation should be vigorously sustained, and they should be presented for transplantation before their general condition has seriously deteriorated. A previous attempted drainage procedure does not adversely affect the results of liver transplantation and portoenterostomy should therefore be attempted in all infants under 120 days at presentation. Over that age primary liver transplantation may be preferable.

This condition, the aetiology of which is unknown, is one of the causes of neonatal cholestatic jaundice. Since surgery in the form of a drainage procedure is usually contraindicated, it is important that it is distinguished from extrahepatic biliary atresia. Biliary hypoplasia presents as an isolated finding diagnosed by liver biopsy or as a syndromic form recognized by the presence of characteristic clinical features and confirmed histologically. The incidence is approximately 1/100000 live births. The major features of the syndromic form, not all of which will be present in each case, are chronic cholestasis, prominent forehead, hypertelorism, saddle nose, pointed chin (Fig. 2) 2153, pulmonary artery branch hypoplasia or stenosis, vertebral arch defects (butterfly vertebrae), and posterior embyrotoxin of the eye. Minor clinical features may include growth retardation and disturbance of mental, gonadal, renal, and/or bone function. Features of the syndrome have been documented in parents and siblings. Because the findings on ultrasound and HIDA scan may be identical to those in extrahepatic biliary atresia the identification of clinical features is a great help to the pathologist. The diagnosis is histological, by exclusion of other conditions: there is a reduction in the number and/or size of intrahepatic bile ducts combined with hypoplasia of the extrahepatic ducts. A wedge liver biopsy provides a more representative sample of tissue. Management centres on support of liver, cardiac, and renal function and maintenance of nutrition. The prognosis is better in patients with the syndromic rather than the non-syndromic form: about 50 per cent die of liver failure in early childhood. There are recent reports that a Kasai type procedure may be beneficial in some cases. Liver transplantation is now an option for those who do badly, but some may not be suitable because of the associated cardiac or pulmonary abnormalities.

This is a very rare condition of unknown aetiology, which usually presents in the first 2 months of life with jaundice, pale stools, and abdominal distension. The perforation occurs at the junction of the cystic and common hepatic duct and free biliary peritonitis usually results, although the bile leakage may be contained within a false capsule. Although rare, the clinical presentation is very typical, and should suggest the diagnosis. It may be confirmed by ultrasound and HIDA scan and aspiration of heavily bile-stained ascitic fluid. Apart from mild elevation of serum bilirubin, the liver function tests are normal.

Laparotomy allows visualization of the perforation. A peroperative cholangiogram via the gallbladder proves that there is free flow into the duodenum. The perforation is repaired with an absorbable suture. A large Penrose drain is placed appropriately and the abdomen closed. Procedures such as cholecystojejunostomy or choledochojejunostomy are not necessary, though a plasty procedure on the sphincter of Oddi may be indicated if the cholangiogram suggests blockage at that level.

This extremely rare condition in which the bile ducts are filled with biliary sludge may occur in infants with cystic fibrosis, or in babies with Rh incompatibility who have suffered massive haemolysis. It has also been reported in infants who are otherwise normal. Ultrasound makes the diagnosis, which is confirmed at surgery. Operative cholangiography demonstrates the sludge, irrigation and evacuation of which usually results in relief of jaundice. Duodenostomy and sphincterotomy have also been used to evacuate the inspissated material. The prognosis is excellent if the infant is otherwise normal.

Choledochal cyst, originally recognized by Douglas in 1852, is a rare anomaly of the biliary tree, characterized by abnormal dilatation of part of the extrahepatic and/or intrahepatic biliary tree. The incidence is in the order of 1: 100000 live births, and it predominantly affects females (80 per cent), 30 to 45 per cent of whom present in childhood. The forms of choledochal cyst are classified according to site and shape of the by dilatation (Fig. 3) 2154. Type I, the most common, is characterized by fusiform dilatation of the common hepatic duct or common bile duct with distal narrowing. In type II there is pedunculated dilatation or cyst formation in the common bile duct. Type III shows dilatation of the intramural portion of the common bile duct in the duodenum, with an appearance similar to that of a ureterocele and called a ‘choledochocele’. Type IV is characterized by multiple cystic dilatations of the intrahepatic and extrahepatic biliary tree.

The aetiology is unclear. Congenital abnormality of the pancreaticobiliary junction, resulting in a common channel permitting reflux of pancreatic juice into the biliary tree, has been demonstrated in some but not all patients.

The classic presentation is pain, jaundice, and upper abdominal mass although few patients present with such a triad. Fever and cholangitis are frequently present. Other symptoms include pancreatitis, perforation, intussusception, secondary changes of liver failure and cirrhosis, or late malignant change in the older patient. Many patients are asymptomatic, and diagnosed only when ultrasound, CT, or MRI is performed for other reasons. Presentation in pregnancy has been recorded, and the widespread use of ultrasound in obstetrics has resulted in its prenatal diagnosis in the infant.

An accurate anatomical diagnosis is achieved by a combination of ultrasound and endoscopic retrograde cholangiopancreatography (Fig. 4) 2155. For technical reasons, this may not be feasible in a very young child, in which case percutaneous cholangiography may be indicated. HIDA scanning is also helpful and CT or MRI should not usually be required.

In patients who are asymptomatic and in whom the diagnosis has been made coincidentally, no treatment may be necessary other than periodic review. The great anxiety and reason for surgical intervention in such patients is the reported increased incidence of malignant change in the lining of the cyst. Patients who are symptomatic require surgical intervention. The current view is that because of the danger of malignancy the lining of the cyst should be removed and drainage established by anastomosis to proximal healthy duct wall, by choledochojejunostomy or hepaticojejunostomy. Sometimes as in type IV disease both the intrahepatic and extrahepatic biliary systems, are so widely affected that a drainage procedure without excision is all that is feasible. Where a Roux loop is used, it should be at least 40 cm in length in the hope of preventing subsequent cholangitis. The patient should be kept under long-term supervision.

Portal hypertension exists when the pressure within the portal venous system is greater than 10 to 12 mmHg (normal range 5–7 mmHg). Traditionally, the causes of portal hypertension are classified into prehepatic, hepatic, and post-hepatic. The reader is referred to Chapter 24 295 for information regarding intrahepatic and post-hepatic (Budd-Chiari syndrome) portal hypertension. Prehepatic portal hypertension is of particular importance in children, in whom the principles of management of portal hypertension applied to adults are not necessarily applicable.

The most common cause of prehepatic portal hypertension is believed to be thrombosis of the portal vein secondary to an episode of neonatal oomphalitis and sepsis. Up to 60 per cent of patients have no clear history of such sepsis, but it is likely that this was either not diagnosed or was treated as a pyrexial illness of unknown cause in a very young infant. In the Western world, prehepatic portal hypertension has become a rare cause of paediatric portal hypertension, possibly because of improved neonatal care and consequent reduction of sepsis. Other less common causes, more likely to be seen in adults, include previous surgery at the porta, pancreatitis, choledochal cyst, and lymph node enlargement. Although the portal vein thrombosis may have occurred in the neonatal period, the diagnosis is often not made until later (5 or 6 years of age) when the child presents with haematemesis and/or melaena due to oesophageal varices. Splenomegaly, an inevitable consequence of portal hypertension, is another mode of presentation. Ascites may be present.

The diagnosis is suggested by the history and results of a physical examination. Ultrasound examination shows a normal liver, with blockage or non-visualization of the portal vein, with or without collaterals, and an enlarged spleen. Doppler studies show the hepatofugal direction of flow in the portal vein; non-visualization of the portal vein is suggestive of the diagnosis. Splenoportography or venous phase superior mesenteric-coeliac axis angiography (either standard or using digital subtraction) are diagnostic, showing no flow, the presence of a block, or cavernous transformation of the portal vein, which represents recanalization of portal vein thrombosis and associated development of collaterals. Demonstration of varices on barium swallow confirms the diagnosis. Liver function is almost always normal in prehepatic portal hypertension. This is in contrast to portal hypertension resulting from post-hepatic and intrahepatic causes, and is of critical importance from a prognostic and therapeutic viewpoint. Provided that the bleeding episodes can be controlled, the prognosis for prehepatic portal hypertension is excellent. The bleeding episodes may be major, but are usually self-limiting. Occasionally they are life-threatening, but fatalities are unusual. The frequency and severity of such bleeds diminish with increasing age at about 15 years with development of collaterals, which reduce the portal venous pressure. Management of haemorrhage is supportive, with blood transfusion, pitressin infusion (0.33 units/kg over 20 min), and sedation.

A triple lumen Sengstaken Blakemore tube (paediatric size 15FG) should be passed without hesitation in patients with life-threatening refractory bleeds. Care must be taken to avoid aspiration. The tube is taped to a spatula and to the cheek for traction or to the cot. Inflation of the stomach balloon with 30 to 50 ml air often stops bleeding. Inflation of the oesophageal balloon, if required, ought not to exceed 30 mmHg, 100 cm air, or pressures quoted by the manufacturers, to avoid mucosal ischaemia. This balloon should be deflated every 24 h to ensure mucosal viability.

The various portosystemic shunts used in the management of adult portal hypertension have all been tried in children, with the exception of portocaval shunt which is not feasible because the portal vein is occluded. Because of the small diameter of the splenic or superior mesenteric vein, splenorenal and mesocaval shunts have a poor long-term patency rate. Although there have been reports of successful shunting in children under 7 years of age, the policy in most centres is to treat each bleeding episode by transfusion, pitressin, and general support, with a Sengstaken tube being used in the most refractory cases. Creation of a shunt should be deferred for as long as possible, preferably until the patient is a teenager or approaching adult size, when there is a greater likelihood of long-term shunt patency, with effective diminution of portal venous pressure and cessation of life-threatening haemorrhage. The effectiveness of sclerotherapy has reinforced this waiting policy.

Endoscopy and sclerotherapy performed by a skilled endoscopist are the treatments of choice for bleeding varices, with a low rebleed rate of less than 10 per cent, low morbidity (10 per cent) and mortality (2.5 per cent). A flexible paediatric sideviewing endoscope is usually used under general anaesthesia or sedation. Repeat injections are performed at fortnightly intervals until obliteration is achieved; this usually requires three or four treatments (range 1–8). Average volumes required per varix are in the order of 0.5 to 2.5 ml of ethanolamine oleate. Ulceration with bleeding, perforation, mediastinitis, and stricture are recognized complications: the last is managed by dilatation. Respiratory complications, with arrhythmias and respiratory distress syndrome may occur during and after the procedure, possibly related to sclerosant injection; many children also experience slight chest discomfort and some develop a low-grade fever. Injection sclerotherapy is successful in most instances: in other patients it controls symptoms until growth permits shunting or the frequency of bleeds lessens as the child approaches adulthood. The value of prophylactic sclerotherapy has not been proven. Emergency surgical devascularizaton of the varices is very rarely necessary nowadays, and is only indicated if all the above treatments fail. Ammonia intoxication and encephalopathy are virtually never seen, because liver function is so good.

This condition, previously thought to be an adult disorder is now documented in neonates and childhood. Two-thirds of the patients also have inflammatory bowel disease. The information therein is applicable to the condition in children.

The development of effective antibiotics has made liver abscess extremely rare. Its aetiology, pathology, and management in children is very similar to that of adults and the reader is referred to Section 22.3 158.

Immunodeficiency disease, particularly chronic granulomatous disease, is probably the most common factor predisposing to the development of liver abscess in young patients. Although rare, it should always be considered if no other cause is evident. The prognosis in this disease is poor. Ultrasound, CT, and HIDA scans allow liver abscess to be diagnosed. Ultrasound-guided needle aspiration will usually yield the causative organism and percutaneous pig-tail catheter drainage under ultrasound or CT guidance is the treatment of choice, and has reduced the mortality and morbidity of liver sepsis.

Cholecystitis and gallstones are rare in children. About 20 per cent of stones that do occur are radio-opaque, and they are four times more common in females than in males. The widespread use of ultrasound has resulted in the more frequent detection of idiopathic stones, which form the great majority. Recognized predisposing factors include haemolytic disorders (bile pigment stones, hereditary spherocytosis), cystic fibrosis (mixed stones), infection (mixed stones), stasis and biliary obstruction (mixed stones), prolonged parenteral nutrition, malabsorption states (impaired enterohepatic circulation), liver disease, and high cholesterol levels. Although cholelithiasis may be asymptomatic, classic features of biliary colic may occur, and in those with fever, leucocytosis and right upper quadrant tenderness, cholecystitis is diagnosed.

The diagnosis is made on the basis of history (presence or absence of underlying cause), nature of pain, signs, ultrasound, HIDA scan, and occasionally endoscopic retrograde cholangiopancreatography. Plain films of the abdomen are useful for demonstrating radio-opaque stones, and oral cholecystography is still performed.

Cholecystectomy is used to treat cholelithiasis with cholecystitis. The indications for exploration of the common bile duct are those which apply to adults. When the gallbladder contains a stone and is not inflamed, there is a choice between simple cholecystotomy and removal of the stone, and cholecystectomy with peroperative cholangiography exploration of the common bile duct. Very rarely, sphincterotomy may be indicated. However, the more conservative approach is recommended. The role of endoscopic shock wave lithotropsy and chemical dissolution of pure cholesterol stones in children is unclear: percutaneous laparoscopic cholecystectomy is currently an attractive possibility.

As a consequence of improved care, the median survival of patients with cystic fibrosis is now 21 years, and some live for more than 30 years. Up to 40 per cent of these long-term survivors have liver and/or biliary disease. The gallbladder is frequently small and contracted, and may contain stones. In patients with abnormal liver function tests, endoscopic retrograde cholangiopancreatography often reveals biliary obstruction, with a stricture or stenosis of the distal common bile duct, relief of which results in improvement of liver function. An active surgical approach is therefore now recommended in such patients. Balloon dilatation, surgical relief of strictures, and drainage of the biliary system by cholecystojejunostomy or choledochojejunostomy are being evaluated and used more frequently.

In occasional patients the severity of liver disease far outweighs that of the bronchopulmonary problems associated with this disorder. In this minority group liver transplantation has a role to play in the treatment of end-stage liver failure. However, only those who are not carriers of Pseudomonas and Staphylococcus aureus are considered suitable candidates, and their prognosis is uncertain.

The causes and management of liver trauma in older children are the same as for adults. Hepatic trauma is a rare complication of a difficult birth (0.01 to 0.001 per cent of deliveries) and is associated with a very high mortality. The injury is usually caused by compression and crushing of the abdomen as the infant descends the birth canal, or by obstetrical force during delivery. It occurs more often during breech than vertex delivery, in those with prenatal hepatomegaly, prematurity or postmaturity, and following aggressive resuscitation of the neonate. The diagnosis is usually considered late in a neonate who is pale, with abdominal distension, and increasing peripheral shut down from vasomotor aspects. There is often no external indicator of the injury, although Cullen's sign at the umbilicus or scrotal ecchymosis may be present, and bruising may highlight the difficult nature of the delivery. Ultrasound may help in the diagnosis and an abdominal tap yields blood.

At laparotomy the peritoneum is full of blood. The hepatic laceration may be difficult to identify as it may be high. Laceration may arise at the junction of the hepatic veins and inferior vena cava, the bleeding being partially tamponaded by clot and intra-abdominal pressure. Despite aggressive attempts to suture the laceration, administration of thrombostatic agents (surgical, Avitene, or fibrin glue), fresh frozen plasma, vitamin K, and fresh whole blood, the mortality rate remains high. Death may be due to delayed rupture of a subcapsular haematoma at 3 or 4 days of age. Haemostatic packing may be useful, followed by reoperation after 24 to 48 h.

Approximately 1 per cent of tumours in children under 15 years of age are primary liver tumours, and these comprise the third most common group of intra-abdominal malignancies in children (Table 1) 568, surpassed only by neuroblastoma and Wilms' tumour. Several liver disorders are associated with hepatic tumours, including metabolic (tyrosinaemia, &agr;&sub1;-antitrypsin deficiency, Wilson's disease) and infective disorders (hepatitis B, primary sclerosing cholangitis).

This embryonal tumour contains hepatic epithelial parenchyma cells of different histological types, and 75 per cent of patients present before the age of 3 years. There is a 2:1 male:female ratio. Abdominal distension, weight loss, pallor, malaise, fever, hepatosplenomegaly, and sometimes osteoporosis with fractures are seen. Precocious puberty may occur due to circulating human chorionic gonadotropin, and &agr;-fetoprotein may be present in the serum. The right lobe is more often affected than the left, and 15 per cent of tumours are multifocal.

This occurs in the older child and adolescent as well as in adulthood. Underlying cirrhosis is common, and the tumour may be multicentric. Presentation is similar to that of hepatoblastoma, but fever is less common. The fibrolamellar variant has a better prognosis than typical hepatocellular carcinoma.

This rare tumour is usually an embryonal rhabdomyosarcoma of the biliary tree and presents at 1 to 9 years of age with jaundice, pruritus, fever, and malaise.

An accurate diagnosis and determination of the intrahepatic extent of the tumour dictates resectability. Biopsy is usually, though not always, necessary unless the diagnosis is obvious on the basis of the other findings, particularly &agr;-fetoprotein estimation. Ultrasound and CT scan may be used, but MRI to date has rarely been necessary. Angiography, with or without &sup9;&sup9;Tc&supm; scintigraphy shows a cold nodule. An intravenous pyelogram is occasionally necessary.

The mainstay of treatment is surgical excision, but only one-third of tumours are suitable for surgical resection as a primary treatment. In the remaining two-thirds, preoperative chemotherapy with Adriamycin, cyclophosphamide, vincristine, and 5-flourouracil usually renders the tumour resectable; approximately 13 per cent of tumours remain inoperable. Lobectomies or extended hepatic resections based on the vascular anatomy described by Couinaud are performed. Vascular invasion does not always preclude resectability. The use of embolization has limited application in reducing viable tumour mass, and has limited benefit as a first step modality of therapy. The results of transplantation for malignant disease of the liver are very poor because of tumour recurrence and metastases following immunosuppression.

Follow-up usually involves postoperative multiple chemotherapy. Surveillance for recurrence and metastases is by ultrasound, CT scan, and measurement of biochemical markers such as human chorionic gonadotropin and &agr;-fetoprotein. The best prognosis is seen when there is no or little mitotic activity on pathological examination, fetal histological type and complete surgical excision. The overall survival rates are of the order of 50 to 60 per cent, although patients with the best prognosis may have a 92 per cent 2-year survival rate.

Benign tumours of the liver include mesenchymal hamartomas, infantile haemangioendothelioma, cavernous haemangioma, focal nodular hyperplasia, and adenomas. The angiomatous malformation is the most common and presents with a mass, cardiac failure, hyperconsumptive coagulopathy, and sometimes jaundice. Ultrasound identifies hyperechoic and hypoechoeic areas with large channels showing marked vascularity on colour flow Doppler and a narrow distal abdominal aorta. Angiography defines the precise abnormality (Fig. 5) 2156. As with all haemangiomatous lesions, spontaneous resolution occurs, but active intervention is sometimes required because of increasing heart failure. A variety of treatments have been proposed, including steroids, cyclophosphamide, embolization or ligation of the hepatic artery or its relevant branch, and resection of the involved lobe.

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