WOFAPS 2025 8th World Congress of Pediatric Surgery

Sello Machaea, Milind Chitnis

Walter Sisulu University

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Introduction

Developmental anomalies of the right hepatic lobe are exceedingly rare and are defined as a volume reduction or the absence of liver tissue of the right lobe without previous disease or surgery. Right liver lobe anomalies are typically incidental findings on ultrasound (US) or computed tomography (CT) while investigating other pathologies. As with other developmental anomalies, right liver lobe may be part of a spectrum of other associated abnormalities such as right diaphragmatic hernia, anomalous gallbladder or portal hypertension. We report a case of right hepatic lobe agenesis associated with portal hypertension.

Method

Case review

Results

Case presentation

3year old boy presented with progressive abdominal distention, passing normal stool and no haematemesis. He had a normal vaginal delivery with an uneventful neonatal period. No previous surgical history of note. On examination, he was anicteric, had a distended abdomen with no distended abdominal vein. He had a palpable splenomegaly and an associated reducible inguinal hernia. Haematological findings showed a HB 7.3g/dl with platelets 81, normal liver function. Negative TORCH screen on serology. US abdomen showed a massive splenomegaly with no caudate or right liver lobe and blind ending portal vein. Bowel loops were also noted in the right hemithorax suggesting a right diaphragmatic hernia. CT abdomen revealed a hypertrophied left liver lobe with a dilated portal vein with no filling defects, a non-dilated biliary system and no visualisation of the right liver lobe. A surveillance oesopho-gastroscope was performed which revealed grade 2 oesophageal varices. He is currently on propranolol.

Conclusion

With developmental anomalies of the right liver lobe, other associated anomalies must be actively investigated so that early management can be initiated