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Purpose hyperammonemic encephalopathy is a potentially fatal condition associated with fibrolamellar hepatocellular carcinoma. The mechanism involved in hyperammonemia in patients with fibrolamellar carcinoma was unclear until a possible physiopathological pathway was recently proposed. An ornithine transcarboxylase dysfunction was suggested as a result of increased ornithine decarboxylase activity induced by c-Myc overexpression. This c-Myc overexpression resulted from Aurora Kinase A overexpression derived from the activity of a chimeric kinase that is the final transcript of a deletion in chromosome 19, common to all fibrolamellar carcinomas. Methods we performed the analysis of the expression of all enzymes involved and tested for the mutation in chromosome 19 in fresh frozen samples of fibrolamellar hepatocellular carcinoma, non-tumor liver and hepatic adenomatosis. Results specific DNAJB-PRKACA fusion protein that results from the recurrent mutation on chromosome 19 common to all fibrolamellar carcinoma was detected only in the fibrolamellar carcinoma sample. Fibrolamellar carcinoma and adenomiomatosis samples presented increased expression of Aurora Kinase A, c-MYC and ornithine decarboxylase when compared to normal liver, while ornithine transcarbamylase was decreased. Conclusion The proposed physiopathological pathway is correct and that overexpression of c-Myc may also be responsible of hyperammonemia in patients with other types of rapidly growing hepatomas. This gives further evidence to apply new and adequate treatment to this severe complication.

Background Minimally invasive hepatectomy has well-known advantages over the traditional open approach. Inherent limitations of laparoscopy make major hepatectomies and the resection of upper and posterior segments a great technical challenge. The robotic approach overcomes most of these limitations, and this technology is most useful in the resection of the deeply located caudate lobe. Methods We describe the robotic caudate lobe resection technical aspects, using the first robotic resection of the caudate lobe to treat a biphenotypic hepatocholangiocarcinoma to illustrate the procedure. We also performed a literature review on the current status of the robotic approach to segment (Sg) 1. Results Technical approach to the robotic caudate lobe resection is described in a patient with uneventful post-operative recovery. Literature review demonstrated only four previous authors reporting the use of this technique. Conclusion We present a step-by-step approach to the hepatic Sg 1 resection by robotic approach.

Pancreatic fluid collections are common pancreatitis complications that frequently require drainage. Endoscopic ultrasound-guided placement of expandable lumen apposing metallic stents has recently emerged as an effective and less invasive treatment option. It is associated with less morbidity, lower costs, and faster clinical recovery than other therapeutic modalities. Nevertheless, this procedure may result in severe complications such as bleeding, buried stent syndrome, and prosthesis dislodgement (with perforation and peritoneal leakage). We performed 108 EUS-guided drainages with lumen apposing metallic stents for the treatment of pancreatic fluid collections with 8 complications and only two cases that required urgent surgical procedures resulting in one fatality. We present this two severe complications submitted to surgical treatment and discuss potential signs of alarm that must be taken under consideration before choosing a treatment modality.

Background Laparoscopic ALPPS (Associating Liver Partition and Portal vein ligation for Staged hepatectomy) has previously been reported but has been the authors' default option since 2015 in patients with small future liver remnant. Methods A retrospective analysis of all consecutive patients undergoing ALPPS at a single referral center was performed using a prospective database from July 2011 to June 2016. Feasibility was studied by assessing conversions. The 90-day mortality and complications were analyzed using a Dindo–Clavien score and the comprehensive complication index. Operative time, blood loss, volumetric growth, and hospital stay were examined. The CUSUM analysis was performed. Results ALPPS was performed in 30 patients, 10 of whom underwent a laparoscopic approach. There was no mortality and no complication grade ≥3A observed in laparoscopic ALPPS. In open ALPPS, 10 of 20 patients experienced complications grade ≥3A (p = 0.006) and one patient died. Liver failure was not observed after laparoscopic ALPPS, but two patients in the open ALPPS group developed complications that precluded the second stage. The total hospital stay was shorter in the laparoscopic ALPPS group. Conclusion Laparoscopic ALPPS is feasible as the default procedure for patients with very small FLR, and it is not inferior to the open approach. Surgeons experienced with complex laparoscopy should be encouraged to use a laparoscopic approach to ALPPS.

Primary mucosal malignant melanomas of the gastrointestinal tract are rare tumors associated to poor prognosis. Primary duodenal involvement by pigmented lesions is even more uncommon, and only a few reports exist in the literature. We report the case of a patient with large primary duodenal melanoma that presented with upper intestinal obstruction and bleeding that was submitted to urgent pancreaticoduodenectomy followed by adjuvant systemic therapy with an oral alkylating agent (temozolomide) plus intravenous cisplatin. The patient presents no signs of recurrence 3 years after the surgery. We consider that radical surgical resection followed by systemic therapy is a safe and effective treatment strategy option for primary mucosal gastrointestinal melanomas.

Background The use of laparoscopic glissonean approach has many potential benefits such as shorter operative times, lower blood loss with low morbidity. Methods The aim with this study was to perform an evaluation of 12 years of our experience with laparoscopic glissonean approach in liver surgery, from a technical standpoint using a prospective database. Anatomical laparoscopic liver resections using hilar dissection and non-anatomical resections were excluded from this study. Results 327 patients (170 females and 157 males) with mean age of 56 years were included. 196 (60%) of procedures were major resections. 65% of procedures were performed in the last 5 years. 208 patients were operated on for secondary lesions. In 38 patients the liver was cirrhotic. Morbidity was 37.3% and 90-day mortality occurred in 2 patients (0.6%). Blood transfusion was necessary in 10.7% of patients. Median hospital stay was 4 days. Conclusions Laparoscopic glissonean approach is a safe and feasible technique. It may be preferred in some clinical situations as it is associated with shorter operative times, lower blood loss, and low morbidity. It is superior to standard laparoscopic hepatectomy when an anatomical resection, especially if a segment or section is to be removed. However, application of this technique requires accurate preoperative tumor localization, identification of potential anatomic pedicle variations, as well as surgeon expertise.

Bile duct injuries after cholecystectomy remain a major concern because their incidence has not changed through the years despite technical advances. This video presents a robotic left hepatectomy and Roux-en-Y hepaticojejunostomy as a treatment for a complex bile duct injury after laparoscopic cholecystectomy. A 52-year-old man underwent laparoscopic cholecystectomy at another institution 8 years previously, which resulted in a bile duct injury. His postoperative period was complicated by jaundice and cholangitis. He was treated with endoscopic retrograde cholangiopancreatography and multiple endoprostheses for 3 years, after which the endoprostheses were removed, and he was sent to the authors’ institution. Computed tomography showed that the left liver had signs of disturbed perfusion and dilation of the left intrahepatic bile duct. The patient was asymptomatic and refused any further attempt at surgical correction of the lesion. He was accompanied for 5 years. Magnetic resonance imaging showed progressive atrophy of the left liver. Finally, 3 months before this writing, he presented with intermittent episodes of cholangitis. A multidisciplinary team decided to perform left hepatectomy with Roux-en-Y hepatojejunostomy via a robotic approach. The left liver was atrophied, and left hepatectomy was performed. Fluorescence imaging was used to identify the right bile duct. At opening of the right bile duct, small stones were found and removed. Antecolic Roux-en-Y hepaticojejunostomy then was performed. The operative time was 335 min. Recovery was uneventful, and the patient was discharged on postoperative day 4. Robotic repair of bile duct injuries is feasible and safe, even when liver resection is necessary. This video may help oncologic surgeons to perform this complex procedure.

Background: The isolate resection of the uncinate process of the pancreas is a rarely described procedure but is an adequate surgery to treat benign and low grade malignancies of the uncinate process of the pancreas. Aim: To detail laparoscopic uncinatectomy technique and present the initial results. Method: Patient is placed in supine position with the surgeon between legs. Three 5-mm, one 10-mm and one 12-mm trocars were used to perform the isolated resection of the uncinate process of the pancreas. Parenchymal transection is performed with harmonic scalpel. A hemostatic absorbable tissue is deployed over the area previously occupied by the uncinate process. A Waterman drain is placed. Result: This procedure was applied to an asymptomatic 62-year-old male with biopsy proven low grade neuroendocrine tumor of the pancreatic uncinate process. A laparoscopic pancreaticoduodenectomy was proposed. During the initial surgical evaluation, intraoperative sonography was performed and disclosed that the lesion was a few millimeters away from the Wirsung. The option was to perform a laparoscopic uncinatectomy. Postoperative period until full recovery was swift and uneventful. Conclusion: Laparoscopic uncinatectomy is a safe and efficient procedure when performed by surgical teams with large experience in minimally invasive biliopancreatic procedures.

Repeat hepatectomy often is required for primary and metastatic tumors. The purpose of this video was to present a robotic repeat hepatectomy for recurrent colorectal metastasis after multiple previous open surgeries. A 64-year-old man underwent open left colectomy complicated by anastomotic leak. He was reoperated for colostomy, which was reverted. One year later, he underwent open metastasectomy. Recently, he presented a recurrence in the right liver, and a robotic right hepatectomy was indicated. This approach used five trocars. The operation began with adhesiolysis. The next step was to dissect and divide the right hepatic artery and the right portal vein. A retrohepatic tunnel is created on the right side of the inferior vena cava for a modified liver hanging maneuver. The liver was pulled upwards and liver transection resumed towards the right hepatic vein. The liver was divided with bipolar forceps under continuous saline irrigation. The right hepatic duct was found inside the liver and was divided. Finally, the right hepatic vein was divided inside the liver parenchyma using a vascular stapler, and robotic right hepatectomy was completed. The operative time for docking was 10 min; adhesiolysis took 90 min while robotic right hepatectomy was completed in 240 min. The Pringle maneuver was not used. Estimated blood loss was 150 mL with no need for transfusion. Recovery was uneventful, and the patient was discharged on the fifth postoperative day. Robotic repeat hepatectomy is feasible and safe in experienced hands and may have some advantages over laparoscopic and open repeat liver resections.

Since the development of the robotic platform, the number of robotic-assisted surgeries has significantly increased. Robotic surgery has gained growing acceptance in recent years, expanding to pancreatic resection. Here, we report a total robotic resection of the uncinate process of the pancreas performed in a patient with a cystic neuroendocrine tumor. To our knowledge, this is the first report of a robotic resection of the uncinate process of the pancreas. A 46-year-old man with no specific medical history was diagnosed with a neuroendocrine tumor after undergoing routine imaging. Biopsy guided by echoendoscopy revealed a well-differentiated neuroendocrine tumor. We decided to perform a robotic resection of the uncinate process of the pancreas after obtaining informed consent for the procedure. According to preoperative echoendoscopy and magnetic resonance imaging, there was a safe margin between the neoplasm and the main pancreatic duct. The technique uses five ports. The duodenum is fully mobilized, and Kocher maneuver is carefully performed. The uncinate process of the pancreas is then identified. The resection of the uncinate process begins with the division of small arterial branches from the inferior pancreaticoduodenal artery in its inferior portion, followed by control of venous tributaries to the superior mesenteric vein. Intraoperative localization of the ampulla of Vater is performed using indocyanine green enhanced fluorescence, thus defining the superior margin of the uncinate process. The pancreatic division is made about 5 mm below its upper margin for safety. Surgical specimen is then retrieved through the umbilical port inside a plastic bag. The raw pancreatic area is covered with hemostatic tissue and drained. The total operation time was 215 min. The docking time was 8 min and console time was 180 min. Blood loss was minimum, estimated at less than 50 mL. The postoperative period was uneventful, except for hyperamylasemia in the drain fluid. The patient was discharged on the 3rd postoperative day. The final pathological report confirmed well-differentiated pancreatic neuroendocrine tumor. Robotic resection of the uncinate process of the pancreas is safe and feasible, providing parenchymal conservation in a minimally invasive setting. Robotic resection should be considered for patients suffering from low-grade pancreatic neoplasms located in this part of the pancreas.

Gallbladder cancer is suspected preoperatively in only 30 % of all patients, while the remaining 70 % of cases are discovered incidentally by the pathologist. The increasing rate of cholecystectomies via laparoscopy has led to the detection of more gallbladder cancers in an early stage, and extended resection with regional lymph node dissection has been suggested. We present a video of a totally laparoscopic liver resection (segments 5 and 4b) with regional lymphadenectomy in a patient with an incidental gallbladder cancer. A 50-year-old woman underwent laparoscopic cholecystectomy, and pathology revealed a T1b gallbladder carcinoma. The patient was referred for further treatment. Contact with the primary surgeon revealed that no intraoperative cholangiogram was performed, and the gallbladder was removed intact, with no perforation, and inside a plastic retrieval bag. Pathology revision confirmed T1b, and positron emission tomography/computed tomography was negative. The multidisciplinary tumor board recommended radical re-resection, and a decision was made to perform a laparoscopic extended hilar lymphadenectomy, along the resection of segments 5 and 4b. Operative time was 5 h, with an estimated blood loss of 240 mL. Recovery was uneventful and the patient was discharged on the fourth postoperative day. Final pathology showed no residual disease and no lymph node metastasis. Laparoscopic resection of liver segments 5 and 4b combined with a locoregional lymphadenectomy of the hepatoduodenal ligament is an oncologically appropriate technique, provided it is performed in a specialized center with experience in hepatobiliary surgery and advanced laparoscopic surgery. This video may help oncological surgeons to perform this complex procedure.

BACKGROUND: Anatomical liver resections are based on some basic technical principles such as vascular control, ischemic area delineation to be resected and maximum parenchymal preservation. These aspects are achieved by the intrahepatic glissonian approach, which consists in accessing the pedicles of hepatic segments within the hepatic parenchyma. Small incisions on well-defined anatomical landmarks are performed to approach the pedicles, making dissection of the hilar plate unnecessary. AIM: Analyze parameters in liver anatomy related to intrahepatic surgical technique to glissonians pedicles, to set the normal anatomy related to the procedure and thereby facilitate the attainment of this technique. METHODS: Anatomical parameters related to the intrahepatic glissonian approach were studied in 37 cadavers. Measurements were performed with precision instruments. Data were expressed as mean±standard deviation. The subjects were divided into groups according to gender and liver weight and groups were compared statistically. RESULTS: Twenty-five cadavers were male and 12 female. No statistically significant difference was observed in virtually all parameters when groups were compared. This demonstrates the consistency of the anatomical parameters related to the intrahepatic glissonian approach. CONCLUSION: The results obtained in this study made possible major technical advances in the realization of open and laparoscopic hepatectomies with intrahepatic glissonian approach, and can help surgeons to perform liver resections by this method. RACIONAL: Ressecções hepáticas anatômicas são baseadas em alguns princípios técnicos básicos, como o controle vascular, delimitação de área isquêmica a ser ressecada e máxima preservação do parênquima. Isto pode ser obtido pelo acesso intra-hepático aos pedículos glissonianos, que consiste em controle dos pedículos dos segmentos dentro do parênquima hepático. Pequenas incisões ao redor da placa hilar, em marcos anatômicos bem definidos, são utilizadas para acesso aos pedículos, tornando desnecessária a dissecção do hilo hepático. OBJETIVO: Analisar parâmetros da anatomia do fígado relacionada com a técnica cirúrgica da abordagem intra-hepática aos pedículos glissonianos, para definir a anatomia normal relacionada ao procedimento e, assim, facilitar a realização desta técnica. MÉTODOS: Parâmetros anatômicos relacionados à abordagem intra-hepática aos pedículos glissonianos foram estudados em 37 cadáveres. As medições foram realizadas com instrumentos de precisão. Os dados foram expressos em média±desvio-padrão. Os indivíduos foram divididos em grupos de acordo com o sexo e peso do fígado e os grupos foram comparados estatisticamente. RESULTADOS: Vinte e cinco cadáveres eram do sexo masculino e 12 do feminino. Não houve diferença estatisticamente significativa em praticamente todos os parâmetros quando os grupos foram comparados. Isto demonstra a consistência dos parâmetros anatômicos relacionadas com a técnica intra-hepática de acesso glissoniano. CONCLUSÃO: Os resultados obtidos neste estudo possibilitaram grandes avanços técnicos na realização de hepatectomias abertas e laparoscópicas com abordagem intra-hepática aos pedículos glissonianos, e pode ajudar cirurgiões a realizar procedimentos seguros e eficazes por este método.

One of the main criticisms of laparoscopic liver resection is that it is difficult, or not possible, to perform liver-sparing resections. Our aim was to present short videos where the intrahepatic Glissonian approach was used to perform anatomical liver segmental resections, instead of a larger operation, to avoid unnecessary sacrifice of the liver parenchyma. We selected six types of anatomical liver resections to exemplify the use of the intrahepatic Glissonian approach to perform segment-oriented liver resections. These types of hepatectomies were used as an alternative to right or left hepatectomy, or as an alternative to extended liver resections. The intrahepatic Glissonian approach was feasible in all cases. The use of anatomical landmarks previously described was essential to reach and control the Glissonian pedicles. Among the liver-sparing resections, we were able to perform right anterior (S5 + S8) and posterior (S6 + S7) sectionectomies, resection of segments 2, 3, and 4, and mesohepatectomy (S4 + S5 + S8). No patient presented postoperative liver failure. Laparoscopic liver-sparing resections are feasible and may be a good alternative to hemihepatectomies or extended liver resections. The use of the intrahepatic Glissonian approach can be useful.

Patient: Male, 31 Final Diagnosis: Fibrolamellar hepatocellular carcinoma Symptoms: Encephalopathy Medication:— Clinical Procedure: — Specialty: Gastroenterology and Hepatology Objective: Rare disease Background: Hyperammonemic encephalopathy is a potentially fatal condition that may progress to irreversible neuronal damage and is usually associated with liver failure or portosystemic shunting. However, other less common conditions can lead to hyperammonemia in adults, such as fibrolamellar hepatocellular carcinoma. Clinical awareness of hyperammonemic encephalopathy in patients with normal liver function is paramount to timely diagnosis, but understanding the underlying physiopathology is decisive to initiate adequate treatment for complete recovery. Case Report: A 31-year-old male with fibrolamellar carcinoma and peritoneal carcinomatosis presented with rapid onset hyperammonemic encephalopathy. Despite usual treatment for hepatic encephalopathy, his hyperammonemia was aggravated. A physiopathological pathway to encephalopathy resulting from hepatocellular dysfunction or portosystemic shunting was suspected and proper treatment was initiated, which resulted in complete remission of encephalopathy. Thus, we propose there is a physiopathology path to hyperammonemic encephalopathy in non-cirrhotic patients with fibrolamellar carcinoma independent of ornithine transcarbamylase (OTC) mutation. An ornithine metabolism imbalance resulting from overexpression of Aurora Kinase A as a result of a single, recurrent heterozygous deletion on chromosome 19, common to all fibrolamellar carcinomas, can lead to a c-Myc and ornithine decarboxylase overexpression that results in ornithine transcarboxylase dysfunction with urea cycle disorder and subsequent hyperammonemia. Conclusions: The identification of a physiopathological pathway allowed adequate medical treatment and full patient recovery from severe hyperammonemic encephalopathy.

Minimal access surgery is moving toward reduced size and fewer ports. The aim of this article is to describe our experience with the intrahepatic Glissonian approach for single-port laparoscopic left lateral sectionectomy.We have performed this procedure on 8 consecutive patients. A transumbilical incision is performed, and a single-incision platform is introduced. The operation begins with ultrasound examination of the liver. Intrahepatic Glissonian access of the portal pedicle from segments 2 and 3 is performed, and the pedicle is divided with a stapler. The liver is transected, and the left hepatic vein is divided with a stapler. A surgical specimen is retrieved through the single umbilical incision. No drains are left in place.The median operative time was 68 minutes, and there was minimal bleeding. The median hospital stay was 1 day. Six patients were operated on for liver adenoma. There was no morbidity or mortality. During follow-up (median, 12 months), no patient developed incisional hernia. The cosmetic appearance of the incision was excellent in all cases.Single-port laparoscopic left lateral sectionectomy is feasible and can be safely performed in specialized centers.

BACKGROUND: Central pancreatectomy is an alternative technique for benign or low-grade malignant tumors. Laparoscopic central pancreatectomy has been rarely performed, with only 48 cases reported in the English literature. The aim of this paper was to review all published cases together with 3 cases from our institution. METHODS: All published articles indexed on PubMed were included. Terms used were "laparoscopic central pancreatectomy" or "laparoscopic middle pancreatectomy." Variables studied were the operative time, the type of reconstruction, indications, the use of robotic or hand assistance, blood loss, transfusion, pancreatic fistula, hospital stay, follow-up, development of exocrine and/or endocrine insufficiency, morbidity, and mortality. RESULTS: A total of 51 patients were identified. Twenty-one patients underwent total laparoscopy (41.2%), 27 required robotic assistance (52.9%), one required hand assistance (1.9%), and there were 2 conversions. In 18 cases (35.3%), pancreatic reconstruction involved a Roux-en-Y pancreatojejunostomy, and in 32 cases, pancreatogastrostomy (62.7%). The mean operative time was 356 minutes. Blood loss was minimal in most cases, and only 1 patient required blood transfusion (1.9%). Mortality was nil, but morbidity was high, mainly because of pancreatic fistula (46%). The mean hospital stay was 13.8 days. All patients underwent laparoscopic central pancreatectomy for benign or low-grade neoplasms. The mean follow-up duration was 19.6 months (range, 2 to 48 mo). No patient presented exocrine or endocrine insufficiency. CONCLUSIONS: Laparoscopic central pancreatectomy is a feasible and useful technique for the removal of tumors located in the neck of the pancreas. There are very few centers performing this operation, and therefore, a literature review was necessary to identify its indications and technical possibilities, and to promote its use.

The knowledge of liver anatomy has led to a rapid evolution based on the intrahepatic distribution of the portal pedicle. One great advance in liver surgery was the used of segment-based liver resections. Techniques based with intrahepatic Glissonian access of portal pedicles were described to safely perform anatomical liver resections. We have earlier described a standardized intrahepatic access to right and left liver segments’ pedicles without hilar dissection for anatomical hepatectomies. To improve the intrahepatic Glissonian technique, we designed a new atraumatic instrument for liver pedicle retrieval based on the anatomical liver landmarks. This new instrument was successfully employed in seventeen consecutive liver resections with minimum blood loss and without any complications related to its use. This new instrument, atraumatic retriever, replaces the right angle dissector or Gray clamp. The new instrument can slide easily and smoothly around Glissonian pedicle with a simple movement. This new instrument is a useful adjunct for performing intrahepatic access for liver resections. It can also be used to compass delicate anatomical structures such as esophagus and major abdominal vessels. The retriever can further be used in other common situations, including access for Pringle maneuver, encircling proximal esophagus during total gastrectomies or esophagectomies, and access for total vascular exclusion of the liver. This instrument can also be adapted to be used for laparoscopic liver resections.

Associating liver partition and portal vein ligation for staged hepatectomy (ALPPS) allows R0 resection even for patients with extremely small future liver remnants. The ALPPS procedure was initially described for two-stage right trisectionectomy. Reversal ALPPS is a denomination in which the future liver remnant is the right posterior section of the liver.A 42-year-old woman with colorectal metastases in all segments except segment 1 underwent chemotherapy with objective response and was referred for surgical treatment. The computed tomography (CT) scan showed a predominance of metastases in the left liver and in the right anterior section. The right posterior section had three metastases. The plan was to perform a laparoscopic reversal ALPPS (left portal vein ligation combined with in situ splitting in a two-stage left trisectionectomy).Three metastases in the right posterior section were resected, followed by liver partition and left portal vein ligature. The CT scan showed a 70 % increase in the future liver remnant. The second stage constituted left trisectionectomy. At laparoscopy after division of adhesions, the left Glissonian pedicle was divided with an endostapler. A stapler also was used to transect the left and middle hepatic veins, and the specimen was removed through a suprapubic incision. The operative times were respectively 5 and 3 h, and the patient was discharged on days 4 and 5, respectively. No blood transfusion or intensive care unit stay was necessary. At this writing, the patient shows no evidence of the disease 18 months after the procedure.Reversal laparoscopic ALPPS is feasible and safe. Laparoscopy is useful for decreasing blood loss and optimizing visualization during liver transection.