Migliore Marco, Giraudo Giorgio, Gianotti Laura, Testa Valentina, Borghi Felice
Department of Surgery, General and Oncologic Surgery Unit, Santa Croce e Carle Hospital, Cuneo, Italy.
Department of Surgery, General and Oncologic Surgery Unit, Santa Croce e Carle Hospital, Cuneo, Italy.
Int J Surg Case Rep. 2021 Jun;83:105976. doi: 10.1016/j.ijscr.2021.105976. Epub 2021 May 13.
Limy bile syndrome (LBS) is an unusual condition in which gallbladder and/or bile ducts are filled with paste-like radiopaque material with a high calcium carbonate content. It can be rarely associated with PTH disorder and hypercalcemia.
A 35-year-old woman presented with epigastric and right hypochondrium pain since a few hours. Similar attacks occurred in the past months soon after a pregnancy with vaginal delivery. Laboratory findings were not significant. The abdominal ultrasound highlighted a micro-lithiasis of gallbladder without complications. Considering the recurrent biliary attacks, laparoscopic cholecystectomy was performed with intraoperative diagnosis of LBS. A subsequent endocrinological screening highlighted a normocalcemic hyperparathyroidism associated with Vitamin D deficiency, likely related to the recent pregnancy and not to LBS.
LBS is a rare condition with not clear etiology, frequently associated with cholelithiasis, of which it shares clinical presentation and potential complications. Diagnosis of LBS is based on abdominal X-ray/computed tomography scan, or it could be an intraoperative finding. The gold standard treatment is represented by laparoscopic cholecystectomy. The pregnancy with its related cholestatic phenotype could facilitate the LBS manifestation. An endocrinological screening should be performed to rule out a concomitant calcium metabolism disorder.
Knowledge of this rare condition could help general surgeons handle it properly.
石灰样胆汁综合征(LBS)是一种罕见病症,其中胆囊和/或胆管充满了含钙碳酸盐含量高的糊状不透射线物质。它很少与甲状旁腺功能紊乱和高钙血症相关。
一名35岁女性自数小时前开始出现上腹部和右季肋部疼痛。在过去几个月中,每次顺产分娩后不久都会出现类似发作。实验室检查结果无明显异常。腹部超声显示胆囊有微小结石,无并发症。考虑到反复发作的胆绞痛,进行了腹腔镜胆囊切除术,术中诊断为LBS。随后的内分泌筛查发现了与维生素D缺乏相关的血钙正常的甲状旁腺功能亢进,可能与近期妊娠有关,而非LBS所致。
LBS是一种病因不明的罕见病症,常与胆石症相关,二者临床表现及潜在并发症相似。LBS的诊断基于腹部X线/计算机断层扫描,也可能是术中发现。金标准治疗方法是腹腔镜胆囊切除术。妊娠及其相关的胆汁淤积表型可能促使LBS的表现出现。应进行内分泌筛查以排除同时存在的钙代谢紊乱。
了解这种罕见病症有助于普通外科医生正确处理。