1993-05-20-14 Liver cyst © Tsao www.thefetus.net/
Leland Tsao, MD*, Philippe Jeanty, MD
*Dept. of Radiology, Vanderbilt University, 21st and Garland, Nashville TN 37232-5316. Ph: 615-343-0595, Fax: 615-343-4890
Synonyms: Non-parasitic, simple, solitary or unilocular hepatic cyst.
Definition: Fluid-filled space within the liver with a fibrous capsule and lined by cuboidal epithelium. Ninety percent are unilocular, and most are in the anterior segment of the right lobe. They range in size from a few millimeters to a few centimeters.
Prevalence: Second most common benign hepatic lesion, occurring in 2.5% of the general population, but only 6 cases have been described in fetuses. M1:F4.
Pathogenesis: Unknown. Cuboidal epithelial lining and location within the portal triad suggest aberrant development of intrahepatic bile ducts with no connection to the normal billiary drainage.
Associated anomalies: Rarely, tuberous sclerosis may lead to cysts within the liver and kidneys. Peutz-Jeghers syndrome and omphalocele have also been reported in association with a simple cyst.
Differential diagnosis: Choledochal cysts, cystic dilatation of intrahepatic bile ducts, bowel duplication, and cystic structures within other abdominal structures.
Prognosis: Benign. These cysts grow slowly, and rare complications may be due to their enlargement, infection, or hemorrhage.
Recurrence risk: None known.
Management: Observation for asymptomatic cysts. Postnatally, symptomatic cysts may be sclerosed with ethanol under ultrasound guidance. Surgical excision may be indicated for refractory cysts.
MESH Liver, cyst BDE 0465 ICD9 751.62 CDC 751.610
The prenatal diagnosis of hepatic cysts is uncommon. We present a case report and review of the literature.
A 23-year-old G1P0 patient was referred for evaluation of a female fetus suspected of hydronephrosis. The patient"s anamnesis was unremarkable. The ultrasound examination was normal except for the finding of an isolated cyst of the anterior segment of the right lobe of the liver. The patient was advised that the finding was probably more a curiosity than an anomaly, and she was rescanned by her referring obstetrician every month thereafter. The cyst grew proportionally to the fetus. The girl, now 4-year-old, is asymptomatic.
Figure 1: The cyst is symmetrical to the stomach.
Figure 2: The cyst is external to the gallbladder.
Once thought to be rare, simple hepatic cysts are now routinely detected in adults with CT, MRI and ultrasound. The overall prevalence in the general population is about 2.5%, increasing with age1. They may be found at any age and are more commonly seen in females. The right lobe of the liver is more often affected3. Only six cases of prenatally diagnosed simple hepatic cysts have been reported in the recent literature5-7,28-29.
Pathogenesis and pathology
While the cause is not known, the classic pathologic description has advanced the hypothesis that such cysts are produced in aberrant bile ducts. This is thought to occur either by inflammatory ductal hyperplasia or by obstruction of the duct, retention of fluid, and subsequent cyst formation. This hypothesis is supported by the cuboidal histologic appearance of the cyst lining3. Recent immunohistochemical research reveals that simple cysts display mucin histochemical characteristics similar to cysts due to polycystic disease4.
Liver cysts are either congenital or acquired. A classification is provided in Table 1.
Table 1: Classification of hepatic cysts
Autosomal dominant polycystic disease
Post-traumatic hematoma or chronic laceration
Parasitic infestation (echinococcal disease)
Tumoral (benign and malignant)
Secondary to hepatic inflammation abscess, focal necrosis, or granulomatous disease
There may be some evidence linking such cysts with tuberous sclerosis and Peutz-Jeghers syndrome, but this is uncertain1,2.
Definitive localization of a cystic structure within the fetal liver is usually possible. Differentiation from a vascular structure is simple with Doppler. As with other relatively benign abdominal cysts, the major importance of such a finding is differentiation from cystic lesions which may require urgent prenatal or neonatal treatment, or which may affect the viability of the fetus (Table 2).
Aside from the six cysts mentioned above, another large cyst was erroneously interpreted as ascites within the abdomen8. If the lesion can be localized to the liver, the differential diagnosis includes dermoid, lymphatic, and choledochal cysts; cystic dilatation of biliary ducts with Caroli"s disease (usually multiple), polycystic liver disease (usually multiple) and enteric duplication3,6,9. Occasionally, it may be technically impossible to definitively localize a lesion to the liver. The differential diagnosis is then broadened to other intra-abdominal cystic structures, as shown in Table 2. In one series of 9 cases of prenatally diagnosed abdominal cysts, six were ovarian, one was a liver cyst, one was a Meckel"s diverticulum, and one represented a bowel duplication7.
Table 2: Differential diagnosis of hepatic cyst.
Lymphatic or dermoid cyst within liver
Cystic renal dysplasia
Collecting system duplication
Mesenteric or omental cyst
There have been several reported cases of complications involving newborn infants, including abdominal distention and respiratory distress8,10-12. One reported case presented as a diaphragmatic hernia13. A neonatal ultrasound examination may be performed to confirm the diagnosis and to provide a baseline for future follow-up and management.
Table 3: Complications of non-parasitic simple hepatic cysts.
Hepatomegaly found on routine examination, or causing pressure symptoms
Pain due to infection or hemorrhage within the cyst
Jaundice due to compression and obstruction of biliary system
Rupture with possible peritonitis
Neoplastic degeneration - squamous cell carcinoma
Therapy and prognosis
Most simple cysts will remain asymptomatic. Occasionally, some may cause complications which require treatment. Possible complications are listed in Table 314,15. The majority of reported cases involve either obstruction of the biliary system or symptoms due to the mass effect of the cyst. Generally, this occurs with very large cysts exceeding 10 cm in diameter. Other presentations, including infection, hemorrhage, or rupture, are less common16-19. There has been one reported case of IVC obstruction secondary to a simple hepatic cyst15. Very rarely, squamous cell carcinoma may arise in a simple cyst. Prognosis in this case is grave, as the carcinoma is usually already in an advanced stage when detected20,21. Percutaneous aspiration under ultrasound guidance can yield information regarding the nature of a cystic hepatic lesion; however, aspiration of a simple cyst will generally result in reaccumulation of cyst fluid. Instillation of sclerosing agents such as ethanol or minocycline after cyst aspiration has been successful in preventing reaccumulation of cyst fluid22,23. Surgical intervention is required for neoplastic cysts and refractory simple cysts. If the cyst contains bilious material, then a Roux-en-Y limb is created for drainage. More commonly, serous cysts may be treated by simply unroofing the cyst so that it drains into the peritoneal cavity24,25. Successful laparoscopic treatment has been reported26,27.
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