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 2006-02-02-15 Multicystic liver disease and pregnancy © Werner www.thefetus.net/

Multicystic liver disease and pregnancy

Heron Werner, MD1,2, Bruno W Alencar, MD2, Luis Fernando Alencar, MD2, Pedro Daltro, MD1,2, Romeu Cortes Domingues, MD1

1 Clínica de Diagnóstico por Imagem (CDPI) 2 Instituto Fernandes Figueira (IFF) – FIOCRUZ - Rio de Janeiro – Brazil

This is a 38-year-old woman, G2P0A1, with a multicystic liver disease. She had her first-trimester miscarriage 2 years ago. In the end of the year, she noted an abdominal enlargement associated with abdominal pain. Two months later, a dilated liver could be detected.  Ultrasonography and magnetic resonance (MRI) confirmed the diagnose of hepatic multicystic disease.

While undergoing the diagnostic tests, the patient got pregnant. Prenatal scans were performed monthly with abdominal scans, MRI and functional hepatic tests (Table 1). At 16 weeks, the patient underwent a ultrasound guided hepatic puncture for decompression  (750ml, from liver segments III, V and VIII). At 24 weeks, the same procedure was done and 630 ml of liquid was removed. The criteria established to indicate the procedure was the abdominal pain and the increase of the uterine volume with consequent hepatic compression.

At 29 weeks, the patient received betamethasone for fetal lung maturation. At 36 weeks of gestation, the patient was complaining of severe dyspnea, abdominal discomfort, and epigastric pain.  A cesarean section was performed with longitudinal infra-umbilical incision. A male baby born, weighing 2645g, with Apgar 6/ 9. Postpartum physical examination detected a right hepatic lobe extending as far as right iliac fossa. Mother and baby were discharged from hospital on the third day. At present, the patient is clinically controlled and without hepatic tests alterations.

Table 1: Functional hepatic tests at the moment of the diagnosis, during prenatal care and postnatal.

Exams 

Diagnosis

Prenatal

Postnatal

 Albumin (mg/dL)

 4.8

 4.7

4.0 

 Alkaline phosphatase (U/L)

 87

 92

154

 Direct bilirubin (mg/dL)

 0.2

 0.3

 0.3

 Indirect bilirubin (mg/dL)

 0.4

 0.5

 0.5

 Glutamic pyruvic (GPT) (U/L)

 70

 58

 46

 Glutamic oxaloacetic (GOT) (U/L)

 21

 18

 28

 Y-glutamyl transpeptidase (U/L)                  

 135  

110 

 85

Ultrasound image of the liver showing multiple cysts. Note the 3D view of the liver with Power Doppler

 3D view of the liver with Power Doppler

Coronal T2 showing hepatic cysts and the fetal abdomen (arrow)

Axial T1 showing liver with multiple cysts.

Coronal T2 showing normal fetal face.

Note the enlargement of the maternal abdomen and the hepatic cysts during the surgery.

The healthy baby.

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