BACKGROUND: Pure squamous cell carcinoma (SCC) of the gallbladder is a rare malignant biliary tract tumor predominantly found in the body and neck of the gallbladder. However, its occurrence in the cystic duct is even rarer. Given its rarity, no established guidelines or consensus currently exist regarding the treatment of pure SCC of the gallbladder. We report an unusual case of SCC originating from the cystic duct with the intent of providing insights into the therapeutic approach for this type of malignancy.
METHODS: A male patient presented to our hospital with acute cholecystitis. Unexpectedly, imaging revealed gallbladder malignancy.
METHODS: Pathologic examination after surgery confirmed SCC of the cystic duct.
METHODS: Despite elevated bilirubin levels, we were able to exclude hilar involvement, enabling radical tumor resection. Intraoperatively, we discovered that the tumor was located in the cystic duct, a site associated with a high likelihood of invasion into neighboring organs. The tumor demonstrated a predominantly exophytic growth pattern, which prompted us to refrain from extending the resection range, thereby striking a balance between complete tumor removal and surgical trauma. We performed liver wedge resection only to ensure a negative resection margin while preserving the anatomical structure to the greatest extent possible. Postoperative recovery was rapid and uncomplicated. Pathological examination confirmed pure SCC, which led us to initiate a regimen of nab-paclitaxel and cisplatin, which is known to be effective in other organ SCCs. Remarkably, the patient experienced a rare and severe posttreatment cardiovascular event. Consequently, we switched the patient to a chemotherapy regimen of gemcitabine and cisplatin, which ultimately yielded positive clinical outcomes.
RESULTS: no evidence of tumor recurrence was observed within 1 year after surgery.
CONCLUSIONS: The diagnosis and therapeutic strategy for rare tumors such as gallbladder SCC should be meticulously tailored based on their unique characteristics to optimize postoperative patient outcomes.

BACKGROUND Lymphangiomas are rare and benign malformations of the lymphatic system. The presentation of intra-abdominal lymphangiomas, especially from within the hepatoduodenal ligament, is rare in the adult population. In this report, we examine a lymphangioma within the hepatoduodenal ligament resulting in biliary obstruction. CASE REPORT A 62-year-old man with surgical history of cholecystectomy presented to the hepatobiliary clinic for a peri-hilar cystic lesion identified on surveillance magnetic resonance imaging (MRI). The patient\'s MRI revealed a 5.5-cm cystic lesion at the peri-hilar region, likely arising from the biliary tree, which had been increasing in size and causing biliary dilatation. The patient underwent an endoscopic ultrasound, showing a 4.3×2.2 cm cystic structure likley arising from the cystic duct stump with internal septation. An endoscopic retrograde cholangiopancreatography (ERCP) was performed and demonstrated no communication between the biliary tree and the cystic lesion. Given the uncertain etiology of the lesion and its obstructive nature, the patient was moved to the operating room for a complete excision. A well-encapsulated cystic lesion was identified between the cystic duct and the common hepatic duct, which did not communicate with the biliary tree. Pathology confirmed the diagnosis of lymphangioma with features of vascular channel proliferation in the background of fibrotic stroma and lymphoid aggregates. The vascular channel proliferation demonstrated positive immunohistochemical staining for D2-40. At 3-year follow-up, there was no evidence of post-resection recurrence. CONCLUSIONS This case represents an acquired lymphangioma occurring as a sequela of cholecystectomy, likely caused by interruption of the lymphatic drainage system secondary to surgical manipulation.

BACKGROUND: Laparoscopic subtotal cholecystectomy (LSTC) is a bailout procedure that is undertaken when it is not safe to proceed with a laparoscopic total cholecystectomy owing to dense adhesions in Calot\'s triangle. The main aim of this review was to investigate the early (≤30 days) and late (>30 days) morbidity and mortality of LSTC.
METHODS: A literature search of the PubMed® (MEDLINE®), Google Scholar™ and Embase® databases was conducted to identify all studies on LSTC published between 1985 and December 2020. A systematic review was then performed.
RESULTS: Overall, 45 studies involving 2,166 subtotal cholecystectomy patients (51% female) were identified for inclusion in the review. The mean patient age was 55 years (standard deviation: 15 years). Just over half (53%) of the patients had an elective procedure. The conversion rate was 6.2% (n=135). The most common indication was acute cholecystitis (49%). Different techniques were used, with the majority having a closed cystic duct/gallbladder stump (71%). The most common closure technique was intracorporeal suturing (53%), followed by endoloop closure (15%). Four patients (0.18%) died within thirty days of surgery. Morbidity within 30 days included bile duct injury (0.23%), bile leak (18%) and intra-abdominal collection (4%). Reoperation was reported in 23 patients (1.2%), most commonly for unresolving intra-abdominal collections and failed endoscopic retrograde cholangiopancreatography to control bile leak. Long-term follow-up was reported in 30 studies, the median follow-up duration being 22 months. Late morbidity included incisional hernias (6%), symptomatic gallstones (4%) and common bile duct stones (2%), with 2% of cases requiring completion of cholecystectomy.
CONCLUSIONS: LSTC is an acceptable alternative in patients with a \"difficult\" Calot\'s triangle.

UNASSIGNED: Surgeons may mistakenly consider the right hepatic duct as cystic duct, ligate, and divide it.
METHODS: A 58-year-old woman presented with right upper quadrant (RUQ) abdominal pain, nausea, and RUQ tenderness, but negative Murphy\'s sign. Common bile duct was 10 mm based on abdominal ultrasound. Common hepatic duct and intrahepatic ducts consist of multiple common bile duct (CBD) stones with sludge and multiple small gallstones. Different diagnostic procedures (Computed tomography (CT) scan, magnetic resonance cholangiopancreatography (MRCP), and endoscopic retrograde cholangiopancreatography (ERCP)) showed the connection of the cystic duct to the right hepatic duct. Balloon sweeping for stones extraction and then laparoscopic cholecystectomy was successfully done.
UNASSIGNED: Radiologic evaluations like MRCP, CT scan, ERCP or sonography before or during the surgery/endoscopic interventions seem logical at least for selected patients.
CONCLUSIONS: Before endoscopic/surgical interventions we need to be sure about the anatomy of biliary tree by a suitable para-clinic evaluation.

BACKGROUND: Neuroendocrine neoplasm (NEN) of the cystic duct (CD) is an extremely rare entity, with misty clinical manifestation and incidental, in most cases, diagnosis. Due to its rarity, several dilemmas arise concerning the optimal treatment of this type of malignancy.
METHODS: We report two cases of histologically confirmed NENs of the CD from our institution. Furthermore, we present a literature review focusing on the treatment type and likelihood of recurrence. The two patients underwent laparoscopic cholecystectomy (CCE) due to cholelithiasis and were both diagnosed with well-differentiated Grade 1 (G1) NEN. The first patient did not undergo further treatment as the surgical margins were clear. Regarding the second patient, complementary resection of the CD remnant was performed since the histopathological diagnosis indicated positive surgical margins. Active postoperative surveillance was suggested, and both patients remain disease-free to date. In the literature, we identified 22 previous cases of NENs of CD. Since there are still no standard guidelines, various surgical plans were adopted, varying from simple CCE to hepatic lobectomy and Roux en Y hepaticojejunostomy. Postoperative surveillance is reported for up to four years. Regardless of the implicated treatment plan, no patient was diagnosed with recurrent malignancy and the mortality rate was very low (1/22).
CONCLUSIONS: We propose that cholecystectomy with ligation of the CD proximal to its junction with the common hepatic duct is an adequate oncological treatment for G1 NENs of the CD. When preoperative or perioperative suspicion for malignancy is made, a frozen section of the CD should be sent for pathological examination to confirm radical resection (R0). Nevertheless, there is a need for further research that could validate our findings. HIPPOKRATIA 2021, 25 (3):141-144.

The morphometry and morphology of the components of extrahepatic biliary tree show extensive variations. A beforehand recognition of these variations is very crucial to prevent unintended complications while performing surgeries in this region. This study was conducted to analyse the configuration of the extrahepatic biliary tree and its possible variations, as well as measure the components that limit the cystohepatic triangle. Articles were searched in major online indexed databases (Medline and PubMed, Scopus, Embase, CINAHL Plus, Web of Science and Google Scholar) using relevant key words. A total of 73 articles matched the search criteria of which 55 articles were identified for data extraction. The length of left and right hepatic duct in majority of studies was found to be > 10 mm. A wide range of diameters of hepatic ducts were observed between 5 and 43 mm. The average length of cystic duct is around 20 mm. The length and diameter of the common bile duct are 50-150 mm and 3-9 mm, respectively. The most frequently observed pattern of insertion of cystic duct into common hepatic duct is right lateral, rarely anterior, or posterior spiral insertion can present. The results of this study will provide a standard reference range which instead will help to differentiate the normal and pathological conditions.

UNASSIGNED: Congenital absence of the cystic duct is one of the rare types of anomalies associated with the extrahepatic biliary tract (EHBT). It is often an incidental finding intraoperatively leading to significant implications during the perioperative period.
METHODS: A 25-year-old lady was admitted for an elective laparoscopic cholecystectomy indicated for recurrent symptoms of right upper quadrant pain with evidence of cholelithiasis on ultrasound. During laparoscopy, the cystic duct could not be identified. After retrieval of the gallbladder, a blind ending orifice resembling an obliterated cystic duct was discovered.
UNASSIGNED: Absence of the cystic duct can result from a congenital or an acquired process. In both cases, they are difficult to diagnose pre-operatively even though magnetic resonance cholangiopancreatography (MRCP) has shown great potential in delineating the EHBT. It confers an increased risk of injury to the surrounding biliary tract during cholecystectomy. Therefore, the surgical approach depends on the surgeon\'s operative competency and knowledge related to EHBT anomalies.
CONCLUSIONS: Definitive treatment for patients with symptomatic absent cystic duct is an open cholecystectomy, given its increased likelihood of iatrogenic morbidity. Nonetheless, it is important to highlight that laparoscopic cholecystectomy may be performed if the surgeon carries sufficient skills.

Adenomyomatous hyperplasia, a common non-neoplastic lesion in the gallbladder, is rarely identified in the extrahepatic bile duct. Typically, these lesions appear as a nodule or mural thickening/elevation. However, in exceptional circumstances, pedunculated/polypoid adenomyomatous lesion occurs in the biliary tract; two cases in the gallbladder and only one case in the common bile duct have been reported. Despite their benign nature, adenomyomatous lesions, especially those with a polypoid appearance, are clinically difficult to exclude a possibility of malignant neoplasms. We describe a case of polypoid-type adenomyomatous lesion of the cystic duct in a 72-year-old man, which was considered as a cystic duct neoplasm preoperatively. Gross examination of the resected specimen revealed that the 9 mm-sized cystic duct polyp. Histologically, the polypoid lesion consisted of glands without atypia, fibrous stroma, smooth muscle bundles, and accompanying stromal inflammation, leading to the diagnosis of benign adenomyomatous lesion. The lesion might be considered as adenomyomatous hyperplasia arising in the valve of Heister, while true nature of the lesion is uncertain. Recognition and accumulating for this rare disease will contribute to better clinical management in the future.

BACKGROUND: It is currently unknown which method of cystic duct closure is most effective at reducing the risk of bile leak after laparoscopic cholecystectomy. The aims of this work were to determine the most common closure methods used in the UK and review available evidence on which method has the lowest risk of bile leak.
METHODS: We conducted an online survey through the Association of Upper Gastrointestinal Surgeons (AUGIS). We also undertook a systematic review using PubMed, EMBASE, MEDLINE and the Cochrane Library for studies that compared different methods for cystic duct occlusion and reported postoperative bile leak.
RESULTS: There was significant variation in practice between consultant surgeons. For routine laparoscopic cholecystectomy metal clips were used most (64%) followed by locking polymer clips (33%) and suture ties (3%). In cases of a dilated cystic duct, preferences were locking polymer clips (60%), suture ties (30%) and metal clips (5%). We included six studies in our review with a total of 8,011 patients. Metal clips were associated with an increased odds of bile leak compared with locking polymer clips (OR 5.66, 95% CI 1.13-28.41, p=0.04) or suture ties (OR 4.17, 95% CI 0.72-24.31, p=0.12). Most studies were retrospective, unlikely to be adequately powered, and vulnerable to selection bias.
CONCLUSIONS: Limited available evidence suggests that metal clips have the highest risk of bile leak, but results are not strong enough to recommend a change in current clinical practice. A trial is now required to determine the best method of cystic duct closure.

Over the past two decades, there have been an increasing number of reports describing a sixth type of choledochal cyst (cystic duct cyst) in adults that was not included in Todani\'s classification. This sixth entity has not yet been systematically reviewed in the literature. We therefore explored this condition in adults from the perspective of the clinical presentation, diagnosis and treatment through a systematic review of the evidence. The final analysis included 33 reported cases, with 55% of them reported in Asia. The mean age was 39.3 years old, with a female-to-male ratio of 1.5:1. Magnetic resonance cholangiopancreatography was accurate in establishing the diagnosis in 69% of cases. Where reported, standard laparoscopic/open cholecystectomy was performed in about 74% of patients, while around 25% of them needed extensive surgery. Associated malignancy was reported in 6.1% of cases, while 28% of patients had co-existent gallstone-related disease. No significant post-operative morbidity or mortality was reported. In this era of emergency laparoscopic cholecystectomy, surgeons should be aware of this rare condition, with the particular understanding that it is associated with gallstone-related disease in a significant number of reported cases.