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Hepatopulmonary Syndrome is More Common in Budd-Chiari Syndrome - Myth or Truth?

Dhruvi Shah, Vishrutha Poojari, Shivangi Tetarbe, Ira Shah
Department of Pediatric Gastroenterology and Hepatology, B J Wadia Hospital for Children, Mumbai, India

Address for Correspondence: Dhruvi Shah, 401, Prabhat Apartment, 2nd Gunpowder lane, Mazgaon, Mumbai-400010. Email: dhruvishah401@gmail.com

Keywords: HPS, BCS, Chronic liver disease, Children, Hypoxia

Clinical Problem:
A 6-year-old male suffering from Budd-Chiari syndrome diagnosed at 5 months of age with a block in two hepatic veins and one block in inferior vena cava (IVC) in December 2020, was lost to follow up for the last three years. He presented in July 2025 with fever for 5 days, abdominal distension for 2 days and one episode of haematemesis along with malena. At presentation, he had heart rate 108/min, respiratory rate 30/min, SpO2 84.1% on room air and blood pressure 79/65 mmHg. Height was 97 cm [z score<-3 SD as per Indian Academy of Pediatrics (IAP)] and weight was 12.5 kg (z score <-3SD as per IAP). There was cyanosis with grade III clubbing with palmar erythema. On abdominal system examination, there was ascites and hepatosplenomegaly. Other systems were normal. Serial laboratory investigations are depicted in Table 1. Upper gastrointestinal (GI) endoscopy showed 4 columns of long length oesophageal varices with red colour sign and 1 column of small calibre varix without red colour sign. He underwent endoscopic band ligation (EVL) of the oesophageal varices. Bubble contrast echocardiogram was suggestive of hepatopulmonary syndrome (HPS). CT abdomen with triple phase angiography showed atrophy of the medial segments and the left lobe of liver with hypertrophy of the remaining segments. Liver was nodular. There was arterial phase hyperenhancement with retention of contrast in the portovenous and delayed phases. There was non opacification of the hepatic veins and extrinsic compression of the upper hepatic IVC suggestive of chronic Budd-Chiari syndrome with chronic liver parenchymal disease multiple mesenteric and extra-hepatic venous collaterals.

Table 1. Serial laboratory investigations

	Day of presentation	Day 5 of hospitalisation
Haemoglobin (g/dl)	6.8	7.8
Total White Count (cells/cumm)	5510	6770
Neutrophil %	71.6	67.4
Lymphocyte%	22	23.4
Platelet count (cells/cumm)	72000	35000
Prothrombin time (secs)	14.5	13.7
INR (international normalised ratio)	1.19	1.12
Total Bilirubin (mg/dl)	0.3	0.7
Direct Bilirubin (mg/dl)	0.3	0.4
SGOT/SGPT (U/L)	160/47	56/24
Alkaline Phosphatase (U/L)	-	98
Total protein (g/dl)	4.9	5.3
Albumin (g/dl)	2.9	3.2
pH	7.428	-
Bicarbonate (mEq/l)	16.9	-
Po2 (mmHg)	47.4	-
Sodium (mEq/l)	134	136
Potassium (mEq/l)	4.8	3.7
Ammonium (mmol/L)	79	-
Blood Urea Nitrogen (mg/dl)	26	-
S. Creatinine (mg/dl)	0.32	0.2
CRP (mg/L)	4.8	-