Hepatic Hydrothorax – Rare Complication in Pediatrics

Keywords

Hepatic Hydrothorax; Crirrhosis.

Case Presentation

9 months old girl was detected right pleural effusion in a periodic examination.

Pre-medical history

Normal delivery, persistent jaundice after birth. When she was 4 months old, she was diagnosed hydrocephalus, and operated to place ventriculoperitoneal shunt. At 6 months of age, she was diagnosed CMV hepatitis/cholestatic jaundice and treated with Gancyclovir.

Examine

She had diminished breath sounds in lower lobar of right lung,no crackle rales, no dyspnea, no cough. A cardiovascular examination was normal. An infectious status was unremarkable. Edema wasn’t noted. Liver was 2 cm and spleen was 1 cm under costal margin. She had cholestatic jaundice with yellow stool, no petechiae, no red spider-like blood vessels on the skin.

Investigations

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Table

Chest-Xray: Right pleural effusion (Figure 1).

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Figure 1. Chest- Xray

Abdomen-Xray: Ventriculoperitoneal shunt valve was too close to the right diaphragm (Figure 2).

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Figure 2: Abdomen-Xray

Cardiovascular ultrasound: Mild tricuspid regurgitation, mild pulmonary hypertension. Cranial CT scan: Mild ventricular dilation.

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Table

Serum protein: 67 g/l, Albumin: 35g/l. Amylase serum: 35 U/L. GOT: 428 U/L, GPT 256 U/L, total bilirubin: 209, direct: 104 mcmol/l. PT: 68%, Ure: 5,3 mmol/l , Creatinin: 80 mcmol/l, blood PCR CMV(-), CMV IgG(+), CMV IgM (-).

Investigations

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Figure 3: Process of the right pleural effusion on ultrasound.

Then she was operated to replace AP shunt (because we suspected that ectopic position of AP valve might cause pleural effusion) and found nodes on the liver surface.

Liver biopsy showed Grade IV cirrhosis (Figure 4).

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Figure 4: Liver biopsy (grade IV cirrhosis).

More investigation Injected 99mTc into patient’s abdomen (Figure 5); 2 hours later, 99mTc was found in right pleural (Figrue 6).

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Figure 5. After injection 99mTc into abdomen.

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Figure 6. 2 hours later: 99mTc in right pleural capacity.

Final diagnosis : Hepatic Hydrothorax Treatment with diuretic (furosemide and spironolactone, sandostatin, salt restrict diet) and she got better.

Discussion

Hepatic hydrothorax is defined as a pleural effusion, in patients with cirrhosis without any other underlying primary cardiopulmonary and pulmonary causes [1]. This complication occurs about 4-10% of patients with cirrhosis [2]. In most cases (85%) hepatic hydrothorax develops on the right side, with 13% of cases occurring on the left side and 2% bilateral [2]. Mechanism is the passage of a large amount of ascites from the peritoneal to the pleural cavity through diaphragmatic defects. Typically, the defects are smaller than 1 cm and tend to occur on the right side, and sometimes, just observed by microscopy [3]. Many studies using 99mTchuman Albumin or 99mTc-sulfur colloid and dye studies that show an unidirectional passage of these markers from the abdominal to the pleural cavity in the first 24 hours after administration [4, 5].

Treatment Salt restrict diet, diuretics (furosemide and spironolactone). If not response to treatment, alternative treatment was octreotide, TIPS procedure, liver transplant [6].

Conclusion

In pediatrics, hepatic hydrothorax is a rare condition. In addition, in this patient, sign and symptoms of cirrhosis weren’t clear. Diagnosis cirrhosis based on liver biopsy more than clinical finding. Therefore, she wasn’t diagnosed and treated probably at the initial time. However, there were still some problem in this patient we haven’t good understood, such as the cause of cirrhosis and relationship between hydrocephalus and hepatitis/cirrhosis.