Suen PY Department of Surgery PMH 11 February 2012

Ms Cheung OL F/62 PMH: HT Past Surgical Hx: nil Social Hx: lives with daughter

C/O: abdominal distension and right upper quadrant discomfort for 5 years with increase in severity in recent 6 months Decrease in appetite weight loss (5 pounds in recent 1 year)

no jaundice, no pallor, no cervical lymphadenopathy Abdomen: grossly distended, hepatomegaly with liver span about 25 cm, smooth edge and no shifting dullness

LFT normal (TB 22 umol/L, ALP 64 U/L, ALT 17U/L, albumin 41 g/L) CEA: 6.1 AFP: 1.89 HbsAg: –ve Hb 12.1g/dL, WBC 4.4x10⁹/L

Bedside USG abdomen: huge cystic lesion in liver USG abdomen in x-ray dept. (19/4/11): a huge liver cyst with well- circumscribed, thin and regular wall, about 24 cm in diameter over left lobe, no other liver mass

CT abdomen (15/8/11): a huge liver cyst (near water density) with size of 24x15x24cm over left lobe with significant mass effect, no significant contrast enhancement in the lesion

Laparoscopic liver cyst fenestration (marsupialization/unroofing) offerred; patient opted for OT Operation done on 23/11/11 Findings: a large left hepatic cyst (ab0ut 25 cm in diameter); about 3 litres of serous fluid inside and drained

Sub-umbilical port made under direct vision with pneumoperitoneum created; 10mm epigastric and 5mm right subcostal ports created Cyst wall punctured and cystic fluid drained Cyst wall partially excised Inner lining of cyst wall cauterized A piece of omentum anchored into cystic cavity

Post-operatively: uneventful Discharged on D5 Followed up 1 mouth later: Well, no more abdominal distension nor discomfort Abdomen: soft and not distended Wound healed Pathology: a single layer of cuboidal epithelium, suggestive of simple hepatic cyst

Simple hepatic cysts (majority) polycystic liver disease Neoplastic cysts (benign or malignant) Traumatic cysts Parasitic (hydatid) cysts Pyogenic cysts

cystic formations of the liver, containing serous fluid, usually not communicating with biliary system

Most common cystic lesions of the liver 2 nd most common incidental findings of benign lesions in the liver after hemangioma prevalence : 5% 90-95% asymptomatic

For asymptomatic, female to male ratio about 1:1 For symptomatic, female to male ratio 9:1 No malignant potential About half of patients have a single cyst, whereas the other half have two or more

Pathology: Lined by a single layer of cuboidal or low columnar epithelium Pathogenesis: regarded as a congenital malformation of aberrant bile duct, usually lost communications with biliary tree and may gradually dilate

Majority : asymptomatic Commonly discovered as incidental finding during radiographic studies for unrelated symptoms or for other diseases Common symptoms: abdominal discomfort, abdominal distension, nausea or vomiting Rare symptoms: fever, sweating, back or shoulder pain

Rare Intra-cystic haemorrhage (most common; sudden onset of increase in abdominal pain or distension ) Spontaneous rupture Infection Biliary compression with obstructive jaundice torsion

Usually diagnosed by USG or CT USG findings of simple hepatic cysts Well-circumscribed Thin and regular wall Homogeneously anechoic No septation, mural nodules or projections

CT findings Well-defined Thin and regular wall Homogenous, hypoattenuated fluid with density similar to water

MRI may be considered when the diagnosis is equivocal Well-defined, thin and regular wall Fluid signal intensity: low on T1-weighted images and high on T2-weighted image No wall enhancement, nodules or projections; and no internal signals

Cyst fluid analysis ( percutaneous fluid aspiration for analysis) may also be considered in cases with difficulty in diagnosis Cytological analysis: acellular fluid and absence of mucin Chemical analysis: normal CEA, CA19.9 and bilirubin level

Neoplastic cysts’ characteristics: Multi-locular,septated Thick irregular wall Mural nodules, projections present Thick fluid Mucinous material in fluid Elevated CEA or CA19.9 in fluid

Rare Cystadenomas or cystadenocarcinoma Most are cystadenomas -A benign cystic tumour with potential malignant transformation to cystadenocarcinoma (very rare) Radiologically : complex cystic lesions

Majority of patients require no treatment, just for observation

Symptomatic condition (most common) Intracystic hemorrhage Diagnostic uncertainty

Simple percutaneous aspiration Percutaneous aspiration followed by injection of a sclerosing agent Fenestration (unroofing or marsupialization) Enucleation (rarely applied)

Percutaneous aspiration associated with very high recurrence rate (75-100%) repeated aspiration can result in cyst infection usually not for definitive treatment

sclerosing agents : ethanol, minocycline hydrochloride, tetracycline hydrochloride Recurrence rate: 20-30% contraindicated if there is communication with biliary tract generally reserved for patients with high operative risk

lowest (5 %) recurrence rate Should be considered and offered for most of symptomatic patients A laparoscopic approach is favoured (lots of evidence demonstrates it’s treatment results equivalent to that of an open approach, while it has the advantages of a laparoscopic surgery)

Laparoscopic approach adopted Resection of a portion of the cyst wall allows drainage into the peritoneal cavity and access to its interior Ablation of remaining inner lining of cyst wall by cauterization will minimize recurrences and the risk of ascites A piece of omentum can be anchored into the cavity of cyst to avoid reformation of cyst

M.F. Hansman et al/ The American Journal of Surgery 181 (2001) Cystic lesion(s) Simple cystic lesion(s) symptomatic simple hepatic cyst(s) Fenest. polycystic liver disease Dominant cysts multiple small cysts Fenest.Resect. asymptomatic Obs. complex cystic lesion(s) Obs. Resect.

1. Making a definitive diagnosis of the nature of the cystic lesion -DDx: simple hepatic cysts/neoplastic cysts/others -Inx: US/CT +/- MRI or cystic fluid analysis 2. Determining whether the patient’s symptoms are related to the cystic lesion or not -careful history taking -relevant investigations or procedures

3. Deciding whether to intervene or not - assessing the severity of symptoms, occurrence of complications, certainty of the diagnosis, pre-morbid state and the operative risks 4. Deciding the treatment modality - laparoscopic fenestration, percutaneous aspiration followed by injection of a sclerosing agent, simple aspiration or enucleation

Current Surgical Therapy by John L. Cameron, 10 th ed. Surgery of the liver and biliary tract by L.H. Blumgart, 3 rd ed. Hansman MF et al: Management and long-term follow-up of hepatic cysts, Am J Surg 181: , 2001 Fabiani P et al: long-term outcome after laparoscopic fenestration of symptomatic simple cysts of the liver, Br J Surg 92: , 2005 Mazza OM et al: Magagement of non-parasitic hepatic cysts, Am J Surg 209: , com