Epignathus is a rare congenital orofacial teratoma infrequently associated with intracranial extension. Intracranial extension of an epignathus indicates a poor prognosis; however, only a small number of such cases have been reported. While there have been some studies reporting cases of epignathus expanding directly into the cranium, others have reported no communication between an epignathus and an intracranial tumor. A fetus at gestational week 27 was suspected of having an epignathus with intracranial tumor as shown by ultrasonographic and magnetic resonance imaging. The fetus was stillborn and an autopsy was performed. An epignathus measuring 12 × 6 × 6 cm and weighing 270 g protruded from the mouth, with its base on the soft palate. An intracranial tumor weighing 14 g was located at the middle intracranial fossa and connected to the epignathus through the right side of the sella turcica. The intracranial tumor was encapsulated, and there was no invasion into the brain. Histologically, both the epignathus and intracranial tumor were immature teratomas, with neural and pulmonary components that were especially immature as compared to those of the internal organs and brain tissues of the fetus. There have been several reports of epignathus and intracranial tumors that did not communicate; therefore, careful evaluation is needed when a fetus is suspected of having an epignathus extending into an intracranial lesion. Our case supports the findings that an epignathus can directly expand into the cranium. Moreover, this is a rare case of an epignathus in which the intracranial lesion was encapsulated and did not invade the brain. These rare but important findings will provide additional, potential therapeutic strategies for gynecologists, neurosurgeons, and pathologists.

Laparoscopic resection of choledochal cyst (CC) has become a popular approach. As the discussion about optimal treatment and technical strategies continues, we aimed to investigate perspectives of IPEG members. An online survey was conducted in 2023 on behalf of the IPEG Research Committee. IPEG members were asked to complete an anonymous questionnaire that included 36 items on the management of CC. 148 members responded to the survey (North America:49/Asia:44/Europe:23/South America:21/Others:11) and 116 completed all questions. Most surgeons (92.5%) operate on less than 5 cases annually. Diagnostic tools of choice were Magnetic Resonance Imaging (MRI, 95.9%) and ultrasonography (US, 74.5%). Regarding fusiform-type CC, operative indications were cyst size greater than 10 mm (68.9%), typical symptoms (78.5%), or anomalous pancreatico-biliary junction (63.8%). In unilateral intrahepatic biliary cysts (type IVa) cases, 81.3% of respondents do not perform a simultaneous liver resection with the initial cyst resection. While 22.0% resect the CC at diagnosis, even if asymptomatic, a larger group of surgeons (41%; 49/118) wait until the infant reaches six months. Intraoperative cholangiography and choledochoscopy are performed routinely by 38.9% and 13.7%, respectively. The majority (52.5%) ligates the common bile duct stump just below the CC. Laparoscopic reconstructions are performed by retrocolic hepatico-jejunostomy (48.3%) or hepatico-duodenostomy (45.8%) at similar rates, but when done open, 71.2% of respondents prefer retrocolic hepatico-jejunostomy. For the laparoscopic anastomosis, interrupted sutures with intracorporeal knot tying were most often utilized (48.3%). Inidividual pediatric surgeons treat a small number of patients with CC each year. Laparosopic and open reconstruction techniques vary, likely due to technical challenges. III.

The usage of near-infrared (NIR) fluorescence imaging with indocyanine green (ICG) has gained popularity in many procedures in pediatric surgery. ICG generates fluorescent light only when it combines with a protein. We herein report a novel technique for detecting pancreaticobiliary maljunction (PBMJ) with co-injection of bile and ICG in laparoscopic choledochal cyst resection and hepaticojejunostomy for a pediatric patient. A 4-year-old girl presented with abdominal pain and intermittent vomiting. Enhanced computed tomography and magnetic resonance cholangiopancreatography showed a 17-mm type Ia choledochal cyst. Definitive PBMJ was not detected preoperatively. Laparoscopic choledochal cyst resection and hepaticojejunostomy were performed using five ports. A percutaneous silicon catheter was inserted into the gallbladder, and bile juice was aspirated. The amylase level of the bile juice was over 3 × 105 IU/L. The aspirated bile juice and ICG were mixed and co-injected into the gallbladder through the catheter. ICG combined with protein in bile juice and generated fluorescent light. Dilated common bile duct and pancreas were detected by NIR fluorescence imaging. This imaging technique was helpful for detecting the dissection margin of the distal side of the choledochal cyst inside the pancreatic tissue and preventing injury of the pancreatic tissue. This is the first case of ICG application for laparoscopic choledochal cyst resection in a pediatric patient. After resection of the choledochal cyst, laparoscopic hepaticojejunostomy was completely performed. Our technique is a safe and low-invasive method of detecting and excising the distal side of the cyst without a risk of radiography and residual bile duct.