Successful outcome with oral sirolimus treatment for complicated lymphatic malformations: a retrospective multicenter cohort study.

Purpose: Sirolimus has emerged as a safe and effective treatment for complicated lymphatic malformations (LMs). We aim to prove the effectiveness and safety of sirolimus as a therapeutic option for patients with complicated LMs. Methods: Fifty-eight patients with complicated LMs treated with sirolimus for at least 6 months at multicenter between July 2018 and January 2023 were enrolled. All patients were administered oral sirolimus starting at 0.8 mg/m2 every 12 hours, with target serum concentration levels of 8-15 ng/mL. Evaluation for clinical symptoms and LMs volume on MRI were reviewed to assess treatment response and toxicities. Evaluation of disease response was divided into 3 values: complete response, partial response (significant, moderate, and modest), and progressive disease. Results: The median age at the initiation of sirolimus treatment was 6.0 years (range, 1 month-26.7 years). The median duration of treatment was 2.0 years (range, 6 months-4.4 years). The most common lesions were head and neck (25 of 58, 43.1%). Forty-six patients (79.3%) demonstrated a reduction in LMs volume on MRI or improvement of clinical symptoms including 2 complete responses. The young age group and the patients who underwent few prior therapies showed better responses. None of the patients had toxicities attributable to sirolimus with a Common Terminology Criteria for Adverse Events grade of ≥3. Conclusion: Oral sirolimus treatment brought a successful outcome without severe adverse effects. It could be the first-line therapy, especially for the young age group of complicated LMs, and an additional option for refractory lesions that did not respond to conventional treatment.

Case report: Portal vein ligation: lessons from patients with PRETEXT III hepatoblastoma in restoring future liver remnant before major hepatectomy and literature review.

Background: We describe three cases involving three patients with PRETEXT III hepatoblastoma invading the hepatic hilum. After portal vein embolization, the patients underwent uncomplicated trisectionectomy. Methods: Medical records between March 2016 and March 2021 were reviewed, and three patients were selected. A literature review of techniques for increasing future liver remnant in children diagnosed with hepatoblastoma was also conducted. Results: All tumors involved the right lobe and hepatic hilum (PRETEXT III). After neoadjuvant chemotherapy, the tumor size decreased, but hilar involvement was unimproved. Right portal vein ligation (RPVL) was performed to increase the left lobe volume. Post-ligation, the remnant liver increased. Liver function was restored to normal levels within 5 days after the hepatectomy. All patients underwent two cycles of adjuvant chemotherapy without tumor recurrence. Conclusions: RPVL can be safely performed before extended hepatic resection in children with a giant hepatoblastoma invading the hepatic hilum. The tumor was completely resected by securing a sufficient margin and increasing the residual liver volume with portal vein embolization. The patients recovered and underwent adjuvant chemotherapy without the deterioration of liver function.

Optimal timing for inguinal hernia repair in premature infants: surgical issues for inguinal hernia in premature infants.

Purpose: We analyzed the timing of inguinal hernia repair in premature infants in the neonatal intensive care unit (NICU) considering recurrence, incarceration, and other complications. Methods: In this multicenter retrospective review, premature infants (<37 weeks) in the NICU diagnosed with inguinal hernia between 2017 and 2021 were segregated into 2 groups based on the timing of inguinal hernia repair. Results: Of 149 patients, 109 (73.2%) underwent inguinal hernia repair in the NICU and 40 (26.8%) after discharge. Preoperative incarceration did not differ, but complications with recurrence and postoperative respiratory insufficiency were higher in the NICU group (11.0% vs. 0%, P = 0.029; 22.0% vs. 5.0%, P = 0.01). Multivariate analysis showed that the significant factors affecting recurrence were preoperative ventilator dependence and body weight of <3,000 g at the time of surgery (odds ratio [OR], 16.89; 95% confidence interval [CI], 3.45-82.69; P < 0.01 and OR, 9.97; 95% CI, 1.03-95.92; P = 0.04). Conclusion: Our results suggest that when premature infants are diagnosed with inguinal hernia in the NICU, inguinal hernia repair after discharge may decrease the odds of recurrence and postoperative respiratory insufficiency. In patients who have difficulty delaying surgery, it is thought that surgery should be performed carefully in a ventilator preoperatively or weighed <3,000 g at the time of surgery.

Case report: Magnetic resonance imaging-based three-dimensional printing for reconstruction of complex cloacal malformations.

Background: Surgical reconstruction of the urinary tract, anus, and vagina is the definitive treatment for cloacal malformation. However, this procedure may be technically challenging in patients with a long common channel (>3 cm), because further reconstructive procedures, such as vaginal replacement or vaginal switch maneuver, may be required. Thus, accurate determination of spatial anatomy is essential during surgical planning. Three-dimensional (3D) reconstruction using rotational fluoroscopy, computed tomography (CT), and magnetic resonance imaging (MRI) has recently been reported to help in determining the relationship between the rectum, vagina, and bladder, and provides a more accurate measurement of the channel length compared to conventional cloacography. MRI-based 3D reconstruction provides substantial information regarding soft tissue structures around the cloaca, including the pelvic floor musculature and anus. Case: A 2-year-old girl with cloacal malformation required reconstructive surgery. Colostomy and cystostomy had been performed on the first day of her life. Preoperative loopogram revealed a cloaca with a long common channel (35 mm) and short urethra (9 mm), single vaginal opening in the bladder neck, and the colon anterior to the vagina with a fistula at the vaginal neck. Because the vagina was too short to be pulled through, 3D printing based on MRI was performed to visualize structural relationships prior to surgical correction. Saline was used for cloacal visualization. Furthermore, endoscopy-assisted urogenital mobilization was performed, and vaginal substitution was performed using the rectum. No postoperative complications were observed. Conclusions: We believe this is the first report of the use of MRI-based 3D imaging and printing, with saline as a contrast agent during surgical planning for correction of cloacal malformation. MRI-based 3D printing is a potentially promising technique for surgical planning of cloacal malformation correction in patients with a long common channel, as it provides detailed information about the surrounding soft tissue structures without exposure to radiation or contrasting agents.

Pediatric living donor liver transplantation for biliary embryonal rhabdomyosarcoma: a case report of a case showing disease-free survival over 2 years.

Biliary rhabdomyosarcoma is a rare tumor, but it is still the most common tumor of the biliary tract in children. We report a case of a 6-year-old boy with biliary embryonal rhabdomyosarcoma and liver metastasis, which were treated with neoadjuvant and adjuvant chemotherapy combined with living donor liver transplantation (LDLT). Initial imaging studies showed a low-attenuation intraductal mass from the left hepatic duct to the intrapancreatic common bile duct with diffuse upstream dilatation of the intrahepatic duct and liver metastasis. Endoscopic biopsy revealed embryonal rhabdomyosarcoma. After tumor size reduction through neoadjuvant chemotherapy, LDLT was planned to remove the tumor completely. A left lateral section graft weighing 330 g was harvested from his 38-year-old mother and the graft-to-recipient weight ratio was 1.94%. Routine pediatric LDLT operation was performed with deep excavation of intrapancreatic distal bile duct. The explant liver showed minimal residual embryonal rhabdomyosarcoma with no lymph node metastasis. The patient recovered uneventfully from LDLT operation. Scheduled adjuvant chemotherapy was performed for 6 months. The patient is doing well without any evidence of tumor recurrence for 26 months after LDLT. In conclusion, liver transplantation could be an effective treatment for unresectable biliary rhabdomyosarcoma in children according to the location of tumor.

Living donor liver transplantation in an infant patient with progressive familial intrahepatic cholestasis along with hepatocellular carcinoma: a case report.

Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive inherited disease requiring liver transplantation (LT). Hepatocellular carcinoma (HCC) is very rare in infants. We present a case of living donor LT using a left lateral section graft performed in a 7-month-old female infant diagnosed with PFIC type II and HCC. No mutation on ABCB11 gene was identified. Because of progressive deterioration of liver function, living donor LT with her mother's left lateral section graft was performed. Pretransplant serum alpha-fetoprotein (AFP) level was increased to 2,740 ng/mL, but HCC was not taken into account because of its rarity. The explant liver showed micronodular liver cirrhosis, multiple infantile hemangiomas and two HCCs of 0.7 cm and 0.3 cm in size. The patient recovered uneventfully from the LT operation. This patient has been regularly followed up with abdomen ultrasonography and AFP measurement every 6 months. The patient has been continually doing well for 8 years after the LT. In conclusion, LT is currently the only effective treatment for PFIC-associated end-stage liver diseases. HCC can develop at the cirrhotic liver of any cause, thus elevation of HCC tumor markers in pediatric patients is an important clue to perform further investigation before LT.

Liver transplantation in pediatric patients with progressive familial intrahepatic cholestasis: Single center experience of seven cases.

BACKGROUNDS/AIMS: Progressive familial intrahepatic cholestasis (PFIC) is an autosomal recessive inherited disease requiring liver transplantation (LT). The objective of this study was to investigate the clinicopathological features and posttransplant courses of seven LT recipients with PFIC. METHODS: This was a retrospective single-center study of patients with PFIC who underwent LT from January 2013 to June 2020. RESULTS: Two and five patients were diagnosed with PFIC type 1 and type 2, respectively. For all seven patients, age of PFIC onset was at birth. Jaundice was present in all cases. Mean pretransplant total and direct bilirubin levels were 16.1 ± 8.1 mg/dL and 12.4 ± 6.2 mg/dL, respectively. Median patient age and body weight at LT were 10 months and 7 kg, respectively. Types of donors were mothers of patients in four and deceased donors in three. All five patients with PFIC type 2 recovered uneventfully. One patient each with PFIC type 1 underwent retransplantation due to graft failure or died due to multi-organ failure. Overall graft and patient survival rates at five years were 66.7% and 83.3%, respectively. Bile salt export pump immunohistochemical staining showed normal canalicular expression in two patients with PFIC type 1, focal loss in two patients with PFIC type 2, and total loss in three patients with PFIC type 2. CONCLUSIONS: LT is currently the only effective treatment for PFIC-associated end-stage liver diseases. It is mandatory to perform regular follow-up due to the risk of complications including steatohepatitis, especially for patients with PFIC type 1.

Usability of intraoperative cine-portogram during liver transplantation in young pediatric patients with biliary atresia.

BACKGROUND: Pediatric patients with biliary atresia (BA) often present liver cirrhosis-associated portal hypertension and portal vein (PV) hypoplasia. For successful liver transplantation (LT), it is essential to maintain sufficient PV inflow through stenosis-free PV reconstruction with effective ligation of collateral veins. The aim of this study was to assess the clinical usability of intraoperative cine-portogram (IOCP) in young pediatric patients who underwent LT for BA. METHODS: Medical records of pediatric patients younger than 10 years who underwent primary LT for BA from 2018 to 2020 were reviewed. RESULTS: A total of 31 patients had undergone Kasai portoenterostomy soon after birth. Their median ages at Kasai portoenterostomy and LT were 1 and 11 months, respectively. Types of LT were living-donor LT in 13, deceased-donor split LT in 15, and deceased-donor whole LT in three patients. PV interposition using an iliac vein homograft was performed in 28 patients receiving partial liver grafts. Side-to-side PV unification venoplasty was performed in three patients undergoing whole LT. All patients underwent ligation of collateral veins. IOCP was performed in 6 (19.4%) patients. Four showed no or faint residual venous collaterals. Collateral vein embolization and endovascular stenting were performed in one patient each. PV insufficiency-free survival rate was 100% at 1 year and 93.8% at 3 years. All patients are currently alive with a median follow-up period of 23 months. CONCLUSIONS: Intraoperative cine-portogram can be a useful method for identification and embolization of residual portosystemic collateral veins in young pediatric patients who undergo LT for biliary atresia.

The clinicopathologic and operative characteristics of patients with small nonfunctioning pancreatic neuroendocrine tumors.

BACKGROUND: Incidentally detected, small nonfunctioning pancreatic neuroendocrine tumors (NF-PNETs) are increasingly diagnosed on imaging modalities. This retrospective investigation evaluated the clinicopathologic characteristics and perioperative and oncologic outcomes in patients with small NF-PNETs undergoing curative resection. METHODS: The medical records of 444 patients who underwent pancreatic resection for NF-PNETs at a single, large-volume institution between January 2000 and December 2018 were retrospectively reviewed. Patients were divided into those with small (≤2 cm) and large (>2 cm) tumors based on the largest tumor diameter on preoperative computed tomography (CT). Outcomes were also evaluated in subgroups of patients with small NF-PNET who did and did not undergo lymphadenectomy. RESULTS: Of the 444 patients with NF-PNETs, 195 (43.9%) had small (≤2 cm) and 249 (56.1%) had large (>2 cm) NF-PNETs. The rate of parenchyma-preserving surgery (14.4% vs. 7.2%, p = 0.014) and the ratio of spleen preservation for left-sided pancreatectomy (65.6% vs. 38.3%, p < 0.001) were higher in the small NF-PNET group. Size on CT >2 cm (p < 0.001, hazard ratio [HR]: 5.836, 95% confidence interval [CI]: 2.474-13.769), presence of perineural invasion (p < 0.001, HR: 3.025, 95% CI: 1.640-5.577), World Health Organization (WHO) Grade 2 (p = 0.007, HR: 2.861, 95% CI: 1.325-6.176), and WHO Grade 3 (p < 0.001, HR: 11.537, 95% CI: 5.282-25.199) were independent predictors of disease-free survival (DFS). DFS did not differ significantly in patients with small NF-PNETs who did and did not undergo lymphadenectomy (p = 0.886). CONCLUSIONS: Assessment of long-term oncologic outcomes suggests that surgical resection may cure small NF-PNETs. Minimally invasive surgery and organ-preserving surgery are acceptable treatment options for select patients with small NF-PNETs. The effect on survival outcomes of lymph node dissection for small NF-PNETs remains unclear.

Therapeutic plasma exchange as an effective salvage measure for post-hepatectomy hepatic failure: A case report.

Major hepatectomy can result in post-hepatectomy hepatic failure (PHHF) and therapeutic plasma exchange (TPE) can be used as a salvage procedure for liver support. We herein present a case of 69-year-old male patient with perihilar cholangiocarcinoma who was successfully managed with salvage TPE. Preoperative portal embolization was performed to reduce the parenchymal resection rate. The extent of surgery was right hepatectomy with partial excision of the ventral portion of the segment IV, caudate lobectomy, bile duct resection and extensive lymph node dissection. No noticeable surgical complications occurred after the operation, but serum total bilirubin level increased gradually and reached 10 mg/dl at 1 month after the operation. At postoperative day 38, total bilirubin level raised to 19.8 mg/dl and prothrombin time deteriorated significantly, thus salvage TPE was started. TPE was performed three times per week for 2 weeks; consequently, the total bilirubin level was maintained below 10 mg/dl. A few days later, a rebound of total bilirubin occurred; accordingly, 2 sessions of TPE were performed additionally. Overall, a total of 8 sessions of TPE were performed. The patient was discharged at 84 days after operation. The total bilirubin level returned to normal at 5 months after operation. This patient is doing well for past 9 months. In Korea, TPE for liver support has been approved by the social health insurance since August 2020. In conclusion, salvage TPE is an effective liver support measure for PHHF, thus we suggest starting TPE if serum total bilirubin level reaches 10 mg/dl after hepatectomy.

Necrosectomy of hepatic left lateral section after blunt abdominal trauma in a patient who underwent central hepatectomy and bile duct resection for perihilar cholangiocarcinoma.

When the liver is divided into the right and left halves after central hepatectomy, a serious injury to the one half of the liver can destroy the ipsilateral half. We report a case showing total necrosis of the hepatic left lateral section (LLS) caused by blunt abdominal trauma in a patient who had undergone central hepatectomy and bile duct resection for perihilar cholangiocarcinoma. A 47-year-old female patient was transferred because of postoperative status following blunt abdominal trauma. Five years before, she had been diagnosed with perihilar cholangiocarcinoma. Since the tumor extent was compatible with Bismuth-Corlette type IV, she underwent central hepatectomy and bile duct resection. After five years, she experienced an industrial safety accident, in which a heavy refrigerator fell over her body. She underwent emergency duodenal diversion surgery with distal gastrectomy and Roux-en-Y gastrojejunostomy. During this surgery, serious ischemic injury of the LLS with occlusion of the left portal vein and hepatic artery was identified, but not treated. After three weeks, LLS necrosectomy with repair of the jejunal limb was done. Postoperative bile leak developed and required supportive care for two months for its healing. She is currently doing well without any physical discomfort four months after the necrosectomy. Our experience with this case suggests that an injury to the afferent jejunal limb requires an individualized treatment strategy including long-standing waiting with effective drainage for spontaneous healing. The experience of this case appears to be theoretically matched with late-stage resection of LLS following central hepatectomy and bile duct resection.

Development of intrahepatic cholangiocarcinoma at the remnant intrahepatic cyst portion 10 years after resection of type IV choledochal cyst.

Complete resection of Todani type IV choledochal cyst (CC) is not possible, because the intrahepatic portion is not resectable. We present a case of intrahepatic cholangiocarcinoma that arose from the remnant CC portion that was located within the liver 10 years after resection. A 59-year-old female patient had undergone resection of type IV CC 10 years ago, leaving large remnant portions of CC at the liver and pancreas. Two and four years after resection of the extrahepatic CC, cholangitis with intrahepatic stones developed hence these episodes were treated with percutaneous transhepatic cholangioscopy. Ten years after the first operation, intrahepatic stones and a new mass were identified in follow-up imaging studies. Because the mass was identified as adenocarcinoma on biopsy, we performed left hepatectomy with redo hepaticojejunostomy. Pathologic examination showed a 4.5-cm-sized moderately differentiated adenocarcinoma arising from the remnant CC with lymph node metastasis. The patient recovered uneventfully and is currently undergoing adjuvant chemotherapy. Our case indicates that the remnant intrahepatic CC can undergo malignant transformation long after resection of CC. Since the intrahepatic CC portion in type IV CC is usually unresectable, wide hepaticojejunostomy and life-long observation with regular imaging study follow-up are highly recommended for prevention and early detection of malignant transformation.