A 36 year old woman presented at week 11 in her fifth pregnancy. She had no significant past medical or surgical history. Her gynaecological history included menarche at the age of 12 years, a menstruation pattern of approximately 28 days, one normal birth and three miscarriages.
At week 21, the patient consulted for abdominal pain. Physical examination found her to be well hydrated with normal skin colour. Liver size was increased and palpated 4 cm below the umbilicus. Right-sided abdominal pain in tensified on palpation. A full blood count and liver function tests were normal. Ultrasound revealed multiple well-defined anechoic cysts distributed throughout the liver parenchyma, the largest of which measured 108 mm (Fig. 1). The kidneys showed normal echostructure. A Doppler study of intrahepatic flow proved normal. Magnetic resonance scan showed a massive polycystic liver with different sized cysts affecting the whole organ (Fig. 2).
A conservative strategy was adopted, with clinical follow up, serial liver function test, symptomatic analgesia, and if necessary, puncture and aspiration of the largest cysts. Subsequent obstetric progress was favourable. Fetal development was normal. There were no complications requiring surgical intervention. Gestation culminated at week 39 and 4 days with spontaneous vaginal birth of a healthy child weighting 3550 g. The puerperium was within normal limits.
After delivery, an increase in the level of pain as well as in the number and volume of cysts was observed. Liver function remained normal. At six months, the patient underwent partial liver resection for symptomatic pain relief. In view of the persistence and progression of the disease, she is currently enrolled in a liver transplant program.
Hepatic polycystic disease has autosomal dominant inheritance. It is characterised by the presence of single or multiple cysts of variable size, which contain 2–1000 mL of liquid. A single patient may simultaneously present both small and large cysts. It is more frequently observed in women, and usually appears around the fifth decade of life, so that coexistence with pregnancy is rare. The clinical course is generally benign, with normal liver function since hepatic structure is conserved.1 There may be an increase of alkaline phosphatase and gammaglutamyltranspeptidase, but bilirubin is normal. When hepatomegalia advances sufficiently to produce symptoms, complications arise due to compression of neighbouring structures. Such complications may include ascites and upper esophageal haemorrhage due to variceal rupture attributed to compression of the intrahepatic portal veins by the hepatic cysts. Some patients develop jaundice as a consequence of choledocal compression. Infection is unusual, although there are reported cases of infection with Escherichia coli where progressive and fatal hepatic failure developed. Another unusual complication is massive intracystic haemorrhage, which may require emergency surgical intervention.2
Treatment generally involves puncture and aspiration of the cysts, combined with ultrasound-guided application of a sclerosing solution, usually alcohol. More aggressive measures, such as surgical cyst removal, are reserved for selected cases because they are not risk-free procedures, and because of the lack of improved outcome. Currently, the only known cure for this disease is liver transplantation, a valid option when all other therapeutic measures have failed.
All the pathological potential of hepatic polycystosis is aggravated in cases of pregnancy. In addition to the direct competition for intra-abdominal space, the general changes occurring in pregnancy may aggravate the disease and represent severe limitations on therapeutic strategies, whether medical or surgical. In the case we present here, despite the increase in hepatic volume, evolution was favourable during the period of gestation. This made it unnecessary to resort to invasive or surgical treatment, a complicated and risky procedure during pregnancy considering that the uterus grows towards and occupies the upper abdomen. We found only one comparable published report.3 In that case, the authors did not provide a MRI or ultrasound image showing the affected liver and fetus simultaneously.
For the birth, we considered that there was no vaginal counterindication. The only theoretical complication was that increased intra-abdominal pressure during labour might induce catastrophic rupture. Thus, strict instructions were given to avoid any manipulation or application of abdominal pressure. Decompression via puncture of cysts larger than 5 cm just before the birth was considered, but finally rejected given the favourable evolution throughout the pregnancy.
During the months following birth, evolution of the disease was unfavourable. Growth occurred in the number and size of the cysts, and pain worsened. The patient's condition made it necessary to perform a partial hepatectomy, and she was included in a liver transplant program. We do not know to what extent pregnancy contributed to the observed rapid evolution of the disease in this case.