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7.3 Pathology of the Liver
Alternatively, ce-US after IV US-CM administration signifi cantly improves US potential to diagnose injuries early, with similar sensitivity as CT and may even visualise active bleeding (BUT: at present US-CMs in paediatrics are off-label only).
7.3.5.1 Liver Haematoma
Simple haematoma or subcapsular haemorrhage is usually less threatening, may become more serious if liver capsule is ruptured and signifi cant free peritoneal fl uid seen (potentially with more or less sedimentation depending on time of investigation). NOTE : Free peritoneal fl uid/blood appearing like complicated ascites can be found anywhere in abdomen and does not necessarily correlate with site of injury, as fl uid redistributes depending on positioning and other phenomena. US Findings Initially same echogenicity as liver parenchyma, potentially disruption of other structures such as vessels or outer contour can aid diagnosis (Fig. 7.13a ). Thereafter appear hyperechoic, eventually turns hypoechoic with more or less complex appear­ance. Persisting hypoechoic posttraumatic lesions indicate seroma/cyst or even biloma (the latter particularly when growing) (Fig. 7.13d ).
a
bc
235
d
Fig. 7.13 Liver trauma: ( a ) Large subcapsular liver haematoma (+ +), reactive ascites and pleural
effusion. ( b ) Liver laceration – initial baseline US scan. ( c ) IV ce-US (CEUS) (same patient as in b ) clearly delineates the liver laceration in early phase. ( d ) Cystic transformation of old liver hae- matoma (seroma – diffi cult to differentiate from biloma)
236
7 Liver and Bile System
7.3.5.2 Contusion
More diffi cult to diagnose, often triangular in shape, located in subcapsular. US and CDS fi ndings Initially diffi cult to see, echogenicity changes from hyperechoic to hypoechoic.
(a)CDS may improve detection of contusions by focal disruption of normal vas­cular architecture with regional rarefaction of vessel colour signals.
7.3.5.3 Laceration
Defi nition Disruption of liver parenchyma and capsule. US Findings Discontinuity of contour may be primary fi nding – despicable even in early stages. IV ce-US (CEUS) will aid early diagnosis (Fig. 7.13b, c ). Often appears hypoechoic, whereas surrounding tissue inhomogenously patchy, with more or less echogenic areas. Local haematoma will coexist (see above). NOTE : Pay particular attention to involvement of major structures such as major bile ducts, central PV or involvement of HVs (may develop occlusion and thrombo­sis causing necrosis of respective liver segment). If extrahepatic hypoechoic collec­tions persist after liver trauma, consider bile leakage and biloma.
7.3.5.4 Haemobilia
Haemorrhage into bile ducts – sludge-like echoes in bile ducts/gall bladder.
7.3.5.5 Associated Diaphragmatic Injury
Rarely diaphragm can be involved. Detectable commonly just by reactive palsy with high position of upper liver border + subphrenic haematoma + reactive pleural effu­sion (common).
If diaphragm ruptured, US may be able to demonstrate herniation of liver or haematoma into thorax. NOTE : Reliable assessment of integrity and continuity of entire diaphragm is impossible by US, particularly on left side.
7.3.5.6 Liver Infarction
Rare traumatic fi nding due to dual blood supply, more common after transplantation or surgery and more common in HA than PV, usually branch occlusion. Potentially also more diffuse after severe shock with diffuse hypoperfusion and hypooxygen­ation of liver. Other causes are systemic vascular or haemolytic disease. US and CDS fi ndings Typically wedge shaped, round to oval hypoechoic area with indistinct margins and peripheral distribution reaching to subcapsular area. Secondary necrosis with cystic structures or even hyperechoic foci representing gas, secondary calcifi cation and bile lakes. CDS – lack of perfusion in affected vessel.
7.3.5.7 Role of US in Liver Trauma
First imaging modality in moderate blunt abdominal trauma – need to perform more than just FAST (“focused abdominal US for trauma”, as performed in emergency
7.3 Pathology of the Liver
237
room for detection of free fl uid). Detailed study in any stable patient can help to reduce need for (or for tailoring) CT. US follow-up after 6–24 h is essential. Ce-US improves lesion detection, particularly in early phase (see Fig. 7.13 ). NOTE : Ce-CT is mandatory (after FAST ) in severe and multiple trauma – US may additionally miss signifi cant injuries of retroperitoneal structures, bowel and spine, as well as chest involvement – US fi ndings may serve as indicator injury.
7.3.5.8 Additional Imaging
Acute setting/severe trauma: Ce-trauma-CT mandatory.
For assessing equivocal lesions in stable patient/follow-up: dynamic ce-CT increasingly replaced by ce-MR for radiation protection; consider ce-US if available.
Abdominal plain fi lm – no utility.
Chest fi lm – usually performed in multiple trauma in ER.

7.3.6 Space-Occupying Liver Lesions

7.3.6.1 Simple Cysts
Commonly congenital, usually an incidental fi nding, rarely asymptomatic.
Other causes: posttraumatic, after (displaced) umbilical vein catheter, after abscess, after surgery, bile leakage and biloma, infectious, biliary malformations/ cysts, cystic/necrotic part of tumour. US Findings Anechoic fl uid, spherical, thin and smooth wall, no parenchymal nodules (Fig. 7.14 ). NOTE : As soon as there are septae, parenchymal areas or thick wall-like mem­branes, other entities such as abscesses, hydatid cyst, epidermoid cyst (potentially with complex content) must be considered (see Fig. 7.15 ).
7.3.6.2 Complicated Cysts
Different causes, postinfectious/posttraumatic/postsurgical or hematogenous/ ascending infl ammatory disease and cystic tumours
Fig. 7.14 Cystic liver lesions. Huge, but otherwise
simple liver cyst
238
ab c d
Fig. 7.15 Complicated liver cysts: ( a ) Complicated liver cyst with sedimentation – probably post-
traumatic. ( b ) Complicated liver cyst after a liver abscess. ( c ) Complicated liver cyst with split wall and septations – a hydatid cyst ( d ) Complex cystiform lesion (+ +) after surgery that proved to be a biloma
7 Liver and Bile System
US and CDS fi ndings Similar to simple cysts, but additionally fl oating echoes, septae, thick wall or paren­chymal nodules (Fig. 7.15 ). Need at least follow-up or additional diagnostic efforts. Sometimes resected/punctured, particularly in suspicion of tumour or for (scleros­ing) therapy.
CDS may fi nd vascularisation of wall, septae or parenchymal nodules.
7.3.6.3 Liver Calcifications
Variety of causes such as after haemorrhage, abscess, bile duct concretion, cholangitis, Caroli syndrome, cystic fi brosis, infl ammatory (viral, particularly fetal infections) and meconium peritonitis (capsular calcifi cations). US usually cannot defi ne aetiology. US and CDS fi ndings
• Echogenic spots of varying size with dorsal shadowing, depending on amount of
calcium/size (Fig. 7.16 )
• May (must not!) twinkle on CDS
• Single or multiple
7.3.6.4 Intrahepatic Gas
Either in vasculature or bile ducts:
• In biliary system: usually ascends from duodenum (e.g. due to distal obstruction,
associated with bile duct pathology), may be associated with infection + second-
ary calcifi cations, may also be transient without any sequelae
• In vessels: gas bubbles commonly within PV system, derive from some condi-
tion in GI tract, (e.g. necrotising entocolitis, severe gaseous distention); may also
be idiopathic US and CDS fi ndings Most commonly air/gas gathers in dependent areas (highest parts of liver – upper ventral areas in supine position). Seen as echogenic foci (with comet-tail-like rever­beration echoes), sometimes fl oating echogenic foci visualised in central PV (Fig.
7.17 ).
7.3 Pathology of the Liver
239
ab
Fig. 7.16 Liver calcifi cation: ( a ) Superfi cial liver calcifi cation with shadowing. ( b ) CDS shows
some twinkling artefact of subcapsular calcifi cation
abc
Fig. 7.17 Liver/portal venous gas: ( a ) Intrahepatic bright spots – in this case due to air in bile
ducts. ( b ) Air spikes in portal liver fl ow spectra (due to NEC). ( c ) Bidirectional “Doppler noise” on spectral analysis – obviously not in portal vein branches – thus aerobilia has to be suggested (note arterial hyperaemia)
CDS (Fig. 7.17b, c ): multidirectional colour signals, similar to twinkling arte- fact. Typical bidirectional spikes on spectral trace, helpful for DDx biliary versus vascular air – intravascular if found within portal vein. NOTE : US – most sensitive method for detecting air, long before visualised on plain fi lm. If found, should prompt thorough US investigation of GI tract.
7.3.6.5 Haemangioma
Not a single entity, different ages of manifestation, different tendencies for sponta­neous regression:
• The classical small haemangioma (adult type, “cavernous” haemangioma; –
remeber new classifi cation = venous vascular malformation) – benign condition.
Rarer in childhood, often starting from puberty. Really a venous malformation.
240
ab
7 Liver and Bile System
dc
Fig. 7.18 Liver haemangioma: ( a ) Adult-type echogenic small liver haemangioma (+ +). ( b , c )
Haemangioendothelioma/giant infantile haemangioma (RICH) – see also Fig. Central calcifi cation ( arrow , d )
1.25 (CE-US).
• The neonatal or infantile haemangioendothelioma/“infantile/congenital haeman-
gioma (CH)” (GLUT1 positive or negative). May be small or large, may disap-
pear (RICH – rapidly involuting CH) or stay/grow (NICH – non-involuting CH).
In large CH α-fetoprotein may be elevated, but usually normal. Rarely malignant
transformation into angiosarcoma may occur. New clasifi cation: infantile/con-
genital hepaitc arterio-venous vascular malformation. US Findings Adult cavernous haemangioma – usually homogenous hyperechoic mass with well­defi ned margins (Fig. 7.18a ). Larger lesions may be more complex, contain calcifi - cations or central fi brosis, thrombosis, necrosis and degeneration. Sometimes peripheral hypoechoic halo and acoustic enhancement.
Infantile type haemangioma – usually small hypoechoic nodules, single or mul­tiple, of varying size arise and grow after birth. CH = congenital hemangioma are present at birth. May change size and echotexture, may even vanish (RICH). The GLUT1 negative form is more consistent with a vascular malformation (not a true congenital haemangioma), usually larger and more complex in appearance (often called haemangioendothelioma – Fig. 7.18b–d ).
7.3 Pathology of the Liver
241
CDS Cavernous adult haemangioma: slow blood fl ow, some peripheral vascularisation may be depictable, particularly using aCDS and Ce-US.
Neonatal CH/haemangioendothelioma/haemangioendotheliomatosis (multiple/ numerous lesions throughout liver): high-blood fl ow with signifi cant shunt and hypervascularity in periphery of lesions – large HA, tapering of abdominal aorta distal to coeliac trunk. Large shunt may cause cardiac failure. Spectral analysis – high-fl ow velocities with high-diastolic fl ow + low RI, potentially arterialisation or signifi cantly increased fl ow in affected draining HV. Depending on size and vascu­larity, may demonstrate central vascularity, particularly using aCDS and ce-US.
Ce-US: typical Iris-phenomena of slow centripetal enhancement, with sustained enhancement in portal phase, as in CT or MRI (see Fig. 1.25 ).
7.3.6.6 Mesenchymal Hamartoma
Benign tumour, commonly in younger children. May have arteriovenous shunts, may be pedunculated and secondarily undergo torsion. May be cystic, sometimes similarities to complex vascular/veno-lymphatic malformations.
Rare malignant transformation, may spontaneously regress. US and CDS fi ndings Typically well-circumscribed multilocular mass with smooth well-defi ned borders, anechoic and cystic spaces and echogenic septae – very mixed US appearance. US is useful for depicting tumours and follow-up; specifi c diagnosis diffi cult. Diagnosis made by dynamic MRI, potentially biopsy/resection.
CDS shows vascularisation and reveals arteriovenous shunting if large feeding/ draining vessels (always confi rm by spectral analysis). DDx: Lymphangioma, fi broadenoma, etc.
7.3.6.7 Focal Nodular Hyperplasia (FNH)
Rare in childhood, more commonly during puberty. Associated with oral contracep­tive use, myelo- and lympho-proliferative syndromes, immune disorders, long-term medication (particularly steroids and antineoplastic agents), rarely sporadic, addi­tionally in Abernethy malformations, in children with hereditary haemorrhagic tel­angiectasia, etc.
Regenerative lesions in noncirrhotic liver. US Findings
• Commonly multiple, well-defi ned, iso-, hypo- or even hyperechoic nodules
within normal parenchyma. Spherical to polygonal nodular structure of varying
size without capsule. Behaves like tumour/space occupying lesion, may com-
press surrounding liver tissue causing pseudocapsule. May not be distinguish-
able from other masses
• Central large vascular pedicle leading to central fi brous “scar”
• Echogenicity: iso- or hypoechoic to liver, more easily seen by mass effect than
by its echogenicity (often subscapular – focal bump in liver surface) or in altered
liver parenchymal echotecture (e.g. fatty liver)
242
7 Liver and Bile System
CDS May reveal central scar, more readily appreciated by aCDS. Ce-US: typical central spoke wheel pattern in early arterial phase by radial vessels, no portal/late phase enhancement (no enhancement of parenchyma in late phase).
7.3.6.8 Hepatic Adenoma
Associated with glycogen-storage disease, Fanconi anaemia, galactosaemia, ste­roids, after chemotherapy. US Findings Usually solitary mass, well circumscribed, encapsulated, iso- to hypoechoic, rarely hyperechoic – with heterogeneous features in haemorrhage, glycogen or fat- storage. Never contains central “vascular” scar. CDS May show peripheral, central or combined fl ow – with random stippled pattern. Ce-US: better differentiation (by excluding FNH-typical central scar appearance), demonstration of homogenous arterial phase enhancement with absence of portal phase enhancement.
7.3.6.9 Fatty Tumours
Angiomyolipoma (in tuberous sclerosis), lipoma US fi ndings Hyperechoic mass, varying degree of acoustic shadowing (Fig. 7.19 ). Without clini- cal history or other imaging (e.g. chemical shift MRI) not to be differentiated from adult type haemangioma.
7.3.6.10 Hepatoblastoma
Most common malignant liver tumour in childhood – different microscopic entities. Variable US appearance.
Associated with Beckwith-Wiedemann syndrome, hemihypertrophy syndromes, etc.
Commonly metastasises to lung, brain and skeleton. Often large and asymptom­atic at presentation, elevated α-fetoprotein levels. Vascular invasion common.
Fig. 7.19 Liver angiomyolipoma. Echoic liver
tumour in child with tuberous sclerosis, consistent with a liver angiomyolipoma
7.3 Pathology of the Liver
ab
Fig. 7.20 Liver tumours: ( a ) Liver tumour (+ +) in a 3-year-old that was proven to be a hepato-
blastoma. Bicolour mode used to improve visualisation of subtle echogenicity differences even in diffi cult scanning conditions. ( b ) Complex, partially cystic liver tumour in 9-year-old girl that proved to be a liver sarcoma
US and CDS fi ndings Commonly large space-occupying lesion with inhomogenous echogenicity without specifi c criteria (Fig. 7.20a ). Secondary regressive changes, haemorrhage, necrosis and potentially calcifi cations. Tend to compress surrounding structures. PV throm­bosis and/or invasion of HV.
CDS nonspecifi c, commonly some large vessels with high-velocity fl ow. Ce-US: marked early arterial enhancement (similarly to carcinoma) with quick washout and no PV enhancement. Vascular invasion, disruption and irregular vascu­lar architecture readily appreciated.
243
7.3.6.11 Hepatocellular Carcinoma
Second most common liver malignancy, usually in older children, associated with pre-existing liver disease (hepatitis B, glycogen-storage disease, tyrosinaemia, hae­mochromatosis, Wilson, α1-antitripsin-defi ciency, other forms of liver cirrhosis). Elevated α-fetoprotein levels; rarely multifocal. US and CDS fi ndings Similar to hepatoblastoma. Typically a large mass, hyperechoic to normal liver – with heterogeneous appearance, calcifi cations or necrosis, even with hypoechoic areas. Fibrous capsule causing hypoechoic rim may be present.
CDS similar to hepatoblastoma. Hypervascularity with high fl ow may be depicted. Ce-US: marked early arterial enhancement (similarly to hepatoblastoma) with quick washout and no PV-phase enhancement. Vascular invasion, disruption and irregular vascular architecture readily appreciated.
Fibrolamillar carcinoma (subtype): normal α-fetoprotein levels, usually solitary.
Otherwise nonspecifi c US fi ndings, not differentiable from classic hepatocellular
carcinoma or hepatoblastoma by US. US-guided biopsy may aid diagnosis.
244
7 Liver and Bile System
7.3.6.12 Hepatic Sarcomas
Embryonal Cell Sarcoma
Undifferentiated, usually large tumour, also called malignant mesenchymoma or hepatic mesenchymal sarcoma. Commonly in school age children. α-fetoprotein normal. US and CDS fi ndings : unspecifi c, large, with cystic spaces + solid areas (Fig. 7.20b ). NOTE : Particularly large tumours with complex cystic spaces and septations must raise suspicion.
Rhabdomyosarcoma
Rare tumour arising from muscle cells (bile ducts, etc.), commonly situated cen­trally (see also Sect. 7.4 ). US and CDS fi ndings unspecifi c. Large, usually hypoechoic, causing early second­ary cholestasis due to its position. No other specifi c signs. CDS is helpful to differentiate vessels from dilated bile ducts with potential intra­ductal tumour growth and invasion.
Angiosarcoma
Rare vascular tumour arising from endothelial cells. US fi ndings: multiple small nodules are more common than large mass, with satel­lite nodules. Otherwise nonspecifi c US fi ndings with heterogeneous mass contain­ing cystic and solid components.
Hepatic Leiomyosarcoma
Malignant mesenchymal tumour. Poor prognosis and early metastases. US fi ndings: Predominantly solid mass with potential secondary haemorrhage, oth­erwise nonspecifi c. CDS does not aid diagnosis.
7.3.6.13 Metastasis
Various malignancies may cause liver metastases, particularly neuroblastoma (stage IV or IV), Wilms tumour and lymphoma. Rare metastases from other primaries (e.g. carcinoid/gonadal tumours). US and CDS fi ndings Either solitary or multiple, large or small nodules. May be hyper-, iso- and hypoechoic. Potentially show target-like appearance. Cysts/degenerative changes may be present. CDS of little value, except for demonstrating patency of adjacent compressed vessels. Ce-US will aid differentiation from other liver lesions by showing typical US-CM enhancement dynamics - as known from CT and MR (see Table 1.4 ).
7.3.6.14 Proliferative Disorders
Particularly after transplantation – hepatic involvement in posttransplantation lym­phoproliferative disease and lymphoma common. US Findings Single or multiple hyperechoic masses with commonly homogenous echotexture, well-defi ned margins. In very small and diffuse involvement – widespread nodular