Muqresh Mohamad Ayham, Zebian Sandrella I, Alkadrou Abdulkarim M, Al-Shoaib Osama, Bin Traiki Thamer
Department of Gastroenterology, Dr. Sulaiman Al-Habib Medical Group, Riyadh, Saudi Arabia.
Department of Surgery, Dr. Sulaiman Al-Habib Medical Group, Riyadh, Saudi Arabia.
Am J Case Rep. 2025 Sep 14;26:e948437. doi: 10.12659/AJCR.948437.
BACKGROUND Hypoplasia of the right hepatic lobe is a rare congenital anomaly often discovered incidentally during imaging or surgery. This anomaly can be challenging during surgery, leading to intraoperative complications. Patient with right hepatic hypoplasia usually live normally without symptoms, but it can be associated with liver cirrhosis, portal hypertension, and gallstones. While the exact pathophysiological relationship between congenital hepatic hypoplasia and cholecystitis remains unclear, a few contributing factors have been proposed. These include mutations in the hepatocyte nuclear factor 1B (HNF1B) transcription, impaired gallbladder function, and gallbladder ischemia due to malposition. CASE REPORT A 70-year-old man presented with gangrenous cholecystitis and concurrent hypoplasia of the right hepatic lobe; a combination not previously documented. The patient, initially asymptomatic, presented with sharp epigastric pain and was diagnosed with acute cholecystitis through abdominal ultrasound. However, computed tomography (CT) imaging showed severe hypoplasia of the right liver lobe with associated compensatory hypertrophy of the left lobe in addition to stones in the gallbladder. Despite normal laboratory results, we think that the anomaly delayed the diagnosis until the development of cholecystitis as a complication. The patient underwent a successful laparoscopic cholecystectomy without converting to open surgery. CONCLUSIONS This case underscores the importance of recognizing anatomical variations when planning surgery. Awareness of right hepatic lobe hypoplasia can aid in timely diagnosis and appropriate management, ultimately reducing surgical risks and complications. Furthermore, we recommend performing CT in case of vague or atypical symptoms of acute cholecystitis, as well as when the gallbladder shows malposition on abdominal ultrasound (US). However, we recommend identifying the anatomical structure during the surgery to avoid complications.
背景 右肝叶发育不全是一种罕见的先天性异常，常在影像学检查或手术过程中偶然发现。这种异常在手术过程中可能具有挑战性，会导致术中并发症。右肝发育不全的患者通常无症状地正常生活，但可能与肝硬化、门静脉高压和胆结石有关。虽然先天性肝发育不全与胆囊炎的确切病理生理关系尚不清楚，但已提出了一些促成因素。这些因素包括肝细胞核因子1B（HNF1B）转录突变、胆囊功能受损以及由于位置异常导致的胆囊缺血。
病例报告 一名70岁男性因坏疽性胆囊炎并发右肝叶发育不全就诊，这种组合此前未见报道。该患者最初无症状，出现上腹部剧痛，经腹部超声诊断为急性胆囊炎。然而，计算机断层扫描（CT）成像显示右肝叶严重发育不全，左叶伴有代偿性肥大，此外胆囊还有结石。尽管实验室检查结果正常，但我们认为这种异常导致诊断延迟，直到胆囊炎作为并发症出现。患者成功接受了腹腔镜胆囊切除术，未转为开放手术。
结论 该病例强调了在规划手术时识别解剖变异的重要性。认识到右肝叶发育不全有助于及时诊断和适当处理，最终降低手术风险和并发症。此外，我们建议在急性胆囊炎症状模糊或不典型时，以及腹部超声（US）显示胆囊位置异常时进行CT检查。然而，我们建议在手术过程中识别解剖结构以避免并发症。
