Comparison of splenic vessel preserving distal pancreatectomy and the Warshaw technique for solid pseudopapillary neoplasm in children.

PURPOSE: Spleen-preserving distal pancreatectomy (SPDP) such as splenic vessel preservation (SVP) for solid pseudopapillary neoplasms (SPNs) in the body or tail of the pancreas in children prevents post-splenectomy infection and hematologic disorders. However, SVP could be technically challenging, and the Warshaw technique (WT) could be an alternative technique that has been reported to be safe and effective in adults. We, therefore, compared the perioperative outcomes of SVP and WT in pediatric patients. METHODS: We retrospectively reviewed the medical records of pediatric patients with SPN who underwent SPDP using SVP or WT between November 2002 and November 2018 at a large-sized tertiary referral center. RESULTS: Twenty-eight patients were included. Sixteen (57.1%) patients underwent SVP and 12 (42.9%) patients underwent WT. There were no significant differences in the baseline characteristics between the two groups. Postoperative complications occurred in 8 patients each in the SVP (50%), and the WT (66.7%) groups. Two (12.5%) in the SVP group and 1 (8.3%) in the WT group required additional intervention. During a median follow-up duration of 49 months, there were no significant differences in the incidence of splenic infarctions or perigastric varices between the two groups. CONCLUSIONS: There were no significant differences in the surgical outcomes between WT and SVP in pediatric patients with SPN. WT could be a safe and feasible alternative technique for SVP in challenging cases.

Prenatal prognostic factors for isolated right congenital diaphragmatic hernia: a single center's experience.

BACKGROUND: Right-sided congenital diaphragmatic hernia (RCDH) is relatively rare compared with left-sided congenital diaphragmatic hernia (LCDH). Clinical data of RCDH, especially with respect to antenatal prediction of neonatal outcome, are lacking. The aim of this study was to report the treatment outcomes of patients with antenatally diagnosed RCDH and to evaluate the predictability of observed-to-expected lung area-to-head circumference ratio (O/E LHR) for perinatal outcomes, focused on mortality or extracorporeal membrane oxygenation (ECMO) requirement. METHODS: We retrospectively reviewed the medical records of newborn infants with isolated RCDH. We analyzed and compared the clinical and prenatal characteristics including the fetal lung volume, which was measured as the O/E LHR, between the survivors and the non-survivors. RESULTS: A total of 26 (66.7%) of 39 patients with isolated RCDH survived to discharge. The O/E LHR was significantly greater in survivors (64.7 ± 21.2) than in non-survivors (40.5 ± 23.4) (P =.027). It was greater in survivors without ECMO requirement (68.3 ± 15.1) than non-survivors or those with ECMO requirement (46.3 ± 19.4; P = .010). The best O/E LHR cut-off value for predicting mortality in isolated RCDH was 50. CONCLUSIONS: The findings in this study suggest that O/E LHR, a well-characterized prognostic indicator in LCDH, could be applied to a fetus with antenatally diagnosed RCDH. A large cohort study is required to verify the association between O/E LHR values and the graded severity of RCDH.

Functional and structural evaluation in the lungs of children with repaired congenital diaphragmatic hernia.

BACKGROUND: To evaluate the long-term functional and structural pulmonary development in children with repaired congenital diaphragmatic hernia (CDH) and to identify the associated perinatal-neonatal risk factors. METHODS: Children with repaired CDH through corrective surgery who were born at gestational age ≥ 35 weeks were included in this analysis. Those who were followed for at least 5 years were subjected to spirometry and chest computed tomography for evaluation of their functional and structural growth. Main bronchus diameters and lung volumes (total, left/right) were measured. According to total lung volume (TLV) relative to body surface area, children were grouped into TLV ≥ 50 group and TLV < 50 group and the associations with perinatal-neonatal factors were analyzed. RESULTS: Of the 28 children (mean age, 6.2 ± 0.2 years) with left-sided CDH, 7 (25%) had abnormal pulmonary function, of whom 6 (87%) showed restrictive patterns. All pulmonary functions except FEF25-75% were worse than those in matched healthy control group. Worse pulmonary function was significantly associated with small head and abdominal circumferences at birth. The mean TLV was 1339.1 ± 363.9 mL and LLV/TLV was 47.9 ± 2.5 mL. Children with abnormal pulmonary function were more likely to have smaller lung volumes. In multivariate analysis, abdominal circumference at birth was significantly associated with abnormal lung volume. CONCLUSIONS: A quarter of children with repaired CDH showed abnormal pulmonary function. Small abdominal circumference at birth was associated with abnormal pulmonary function and lower TLV. .

Rapid estrogen receptor-α signaling mediated by ERK activation regulates vascular tone in male and ovary-intact female mice.

Estrogen has been shown to affect vascular reactivity. Here, we assessed the estrogen receptor-α (ERα) dependency of estrogenic effects on vasorelaxation via a rapid nongenomic pathway in both male and ovary-intact female mice. We compared the effect of a primary estrogen, 17ß-estradiol (E2) or 4,4',4″-(4-propyl-[1H]pyrazole-1,3,5-triyl)tris-phenol (PPT; selective ERα agonist). We found that E2 and PPT induced greater aortic relaxation in female mice than in male mice, indicating ERα mediation, which was further validated by using ERα antagonism. Treatment with 1,3-bis(4-hydroxyphenyl)-4-methyl-5-[4-(2-piperidinylethoxy)phenol]-1H-pyrazole dihydrochloride (MPP dihydrochloride; ERα antagonist) attenuated PPT-mediated vessel relaxation in both sexes. ERα-mediated vessel relaxation was further validated by the absence of significant PPT-mediated relaxation in aortas isolated from ERα knockout mice. Treatment with a specific ERK inhibitor, PD-98059, reduced E2-induced vessel relaxation in both sexes but to a lesser extent in female mice. Furthermore, PD-98059 prevented PPT-induced vessel relaxation in both sexes. Both E2 and PPT treatment activated ERK as early as 5-10 min, which was attenuated by PD-98059 in aortic tissue, cultured primary vascular smooth muscle cells (VSMCs), and endothelial cells (ECs). Aortic rings denuded of endothelium showed no differences in vessel relaxation after E2 or PPT treatment, implicating a role of ECs in the observed sex differences. Here, our results are unique to show estrogen-stimulated rapid ERα signaling mediated by ERK activation in aortic tissue, as well as VSMCs and ECs in vitro, in regulating vascular function by using side-by-side comparisons in male and ovary-intact female mice in response to E2 or PPT. NEW & NOTEWORTHY Here, we assessed the estrogen receptor-α dependency of estrogenic effects in vasorelaxation of both male and ovary-intact female mice by performing side-by-side comparisons. Also, we describe the connection between estrogen-stimulated rapid estrogen receptor-α signaling and downstream ERK activation in regulating vascular function in male and ovary-intact female mice.

Incidence and management of oesophageal ruptures following fluoroscopic balloon dilatation in children with benign strictures.

OBJECTIVES: The purpose of this study is to investigate the incidence and management of oesophageal ruptures following fluoroscopic balloon dilatation (FBD) in children with benign oesophageal strictures. METHODS: Sixty-two children with benign oesophageal strictures underwent FBDs. Oesophageal rupture was categorized as intramural (type 1), transmural (type 2), or transmural with free leakage (type 3). The possible risk factors for oesophageal ruptures were analyzed. RESULTS: One hundred and twenty-nine FBDs were performed in these patients. The oesophageal rupture rate was 17.1 % (22/129). The majority (21/22) of ruptures were type 1 and type 2, both were treated conservatively. Only one patient had a type 3 rupture and underwent oesophagoesophagostomy. The patient gender, age, and the length and cause of the stricture showed no significant effect on the rupture (P > 0.05). However, for the patients ≤2 years old, the initial balloon with a diameter ≥10 mm showed a higher oesophageal rupture rate than those <10 mm during the first session (P = 0.03). CONCLUSIONS: Although the oesophageal rupture rate in children was 17.1 %, the type 3 rupture rate was 0.8 %, which usually requires aggressive treatment. For children ≤2 years old, the initial balloon diameter should be <10 mm in the first session for decreasing the risk of oesophageal rupture. KEY POINTS: • The oesophageal rupture rate following balloon dilatation in children was 17.1 %. • The incidence of transmural rupture with free leakage is very low. • Only transmural rupture with free leakage needs aggressive treatment. • For children ≤2 years, the initial balloon diameter should be <10 mm.

Treatment Efficacy and Safety of Ethanol Ablation for Thyroglossal Duct Cysts: A Comparison with Surgery.

OBJECTIVE: To compare the efficacy and safety of ethanol ablation (EA) for thyroglossal duct cyst (TGDC) against surgery. METHODS: This study included 345 patients (289, surgery; 56, EA) treated for TGDC at four institutions between May 2005 and June 2014. Surgery (whole surgery and Sistrunk operation which is the current standard surgical method) and EA were compared with respect to the treatment failure and complication rates. The cost of EA and surgery was also compared. The Cox regression hazard model and linear regression were used for the adjustment of covariates. RESULTS: EA demonstrated higher treatment failures (19.6% vs. 2.4%, p < 0.001[whole surgery] and 1.2%, p = 0.004 [Sistrunk operation]), but fewer complications (1.8% vs. 10.0%, p = 0.04 [whole surgery] and 10.2%, p = 0.06 [Sistrunk operation]), and lower cost (₩423,801 vs. ₩1,435,707 [whole surgery]) than surgery. EA achieved 85.7% of treatment success up to second session. The mean volume reduction rate after EA was 82.3% at last follow-up. Young age and EA were correlated with treatment failure (p = 0.01 and 0.001, respectively). CONCLUSION: Both surgery and EA had acceptable treatment efficacy in the management of TGDC. Although there is a higher likelihood of treatment failure with EA, it has a better safety profile than surgery. KEY POINTS: • Both surgery and ethanol ablation show acceptable treatment efficacy for TGDC. • Considering treatment failure, surgery manages TGDC more effectively than EA. • EA is safer than surgery and presents no major complications. • EA could be an alternative treatment for TGDC in selected patients.

All-trans-retinoic acid ameliorates hepatic steatosis in mice by a novel transcriptional cascade.

UNLABELLED: Mice deficient in small heterodimer partner (SHP) are protected from diet-induced hepatic steatosis resulting from increased fatty acid oxidation and decreased lipogenesis. The decreased lipogenesis appears to be a direct consequence of very low expression of peroxisome proliferator-activated receptor gamma 2 (PPAR-γ2), a potent lipogenic transcription factor, in the SHP(-/-) liver. The current study focused on the identification of a SHP-dependent regulatory cascade that controls PPAR-γ2 gene expression, thereby regulating hepatic fat accumulation. Illumina BeadChip array (Illumina, Inc., San Diego, CA) and real-time polymerase chain reaction were used to identify genes responsible for the linkage between SHP and PPAR-γ2 using hepatic RNAs isolated from SHP(-/-) and SHP-overexpressing mice. The initial efforts identify that hairy and enhancer of split 6 (Hes6), a novel transcriptional repressor, is an important mediator of the regulation of PPAR-γ2 transcription by SHP. The Hes6 promoter is specifically activated by the retinoic acid receptor (RAR) in response to its natural agonist ligand, all-trans retinoic acid (atRA), and is repressed by SHP. Hes6 subsequently represses hepatocyte nuclear factor 4 alpha (HNF-4α)-activated PPAR-γ2 gene expression by direct inhibition of HNF-4α transcriptional activity. Furthermore, we provide evidences that atRA treatment or adenovirus-mediated RAR-α overexpression significantly reduced hepatic fat accumulation in obese mouse models, as observed in earlier studies, and the beneficial effect is achieved by the proposed transcriptional cascade. CONCLUSIONS: Our study describes a novel transcriptional regulatory cascade controlling hepatic lipid metabolism that identifies retinoic acid signaling as a new therapeutic approach to nonalcoholic fatty liver diseases.

TRIM72 is required for effective repair of alveolar epithelial cell wounding.

The molecular mechanisms for lung cell repair are largely unknown. Previous studies identified tripartite motif protein 72 (TRIM72) from striated muscle and linked its function to tissue repair. In this study, we characterized TRIM72 expression in lung tissues and investigated the role of TRIM72 in repair of alveolar epithelial cells. In vivo injury of lung cells was introduced by high tidal volume ventilation, and repair-defective cells were labeled with postinjury administration of propidium iodide. Primary alveolar epithelial cells were isolated and membrane wounding and repair were labeled separately. Our results show that absence of TRIM72 increases susceptibility to deformation-induced lung injury whereas TRIM72 overexpression is protective. In vitro cell wounding assay revealed that TRIM72 protects alveolar epithelial cells through promoting repair rather than increasing resistance to injury. The repair function of TRIM72 in lung cells is further linked to caveolin 1. These data suggest an essential role for TRIM72 in repair of alveolar epithelial cells under plasma membrane stress failure.

Laparoscopic distal pancreatectomy to treat solid pseudopapillary tumors in children: transition from open to laparoscopic approaches in suitable cases.

PURPOSE: The aim of this study was to evaluate the outcomes of open and laparoscopic distal pancreatectomy (LDP) in the treatment of solid pseudopapillary tumors (SPT) in children. METHODS: This was a retrospective study of 22 patients under 18 years of age who underwent a distal pancreatectomy for SPT between January 1995 and December 2012. RESULTS: Fourteen patients and eight patients underwent LDP and open distal pancreatectomy (ODP), respectively, and 71.4 % of the LDP and 25.0 % of the ODP procedures were spleen-sparing operations. The median duration of surgery in the LDP group was shorter than that in the ODP group [175 (range 120-540) vs. 257 (range 200-305) min, p = 0.024]. There were no differences in postoperative complications. The LDP patients commenced oral intake earlier than the ODP patients [2.0 (range 1.0-7.0) vs. 4.0 (range 3.0-12.0) days, p = 0.010], and had an earlier discharge from hospital [7.0 (range 5.0-20.0) vs. 13.0 (range 7.0-22.0) days, p = 0.009]. CONCLUSION: LDP treatment for SPT in children is associated with a shorter hospitalization and a shorter time to oral intake compared to ODP. LDP is a safe and feasible option for SPT in select pediatric patients.

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.

An idiopathic congenital abdominal aortic aneurysm with impending rupture in a 23-month-old boy.

Abdominal aortic aneurysms are distinctly uncommon in infants and children. These aneurysms, which are idiopathic in nature without any definite predisposing factors, are exceedingly rare. We present the case of a giant idiopathic congenital infrarenal abdominal aortic aneurysm with impending rupture in a 23-month-old boy, which was successfully treated with surgical repair using a cryopreserved cadaveric allograft. To the best of our knowledge, this is the oldest case and the third successful treatment of an idiopathic congenital abdominal aortic aneurysm repaired with a cryopreserved allograft in infants and children. Continued follow-up with multimodality imaging is required.

Shifting from laparotomy to thoracoscopic repair of congenital diaphragmatic hernia in neonates: early experience.

BACKGROUND: Thoracoscopic repair of congenital diaphragmatic hernia (CDH) is now widely performed by pediatric surgeons. We compare our experience with this operation versus the results for a group of CDH patients treated by laparotomy at the same center over the same period. METHODS: From January 2008 to December 2011, we reviewed the medical records of 50 neonates who underwent surgery for posterolateral CDH (34 by laparotomy, 16 by thoracoscopy). Two thoracoscopic operations (12.5 %) were converted to thoracotomy. Eight patients treated with extracorporeal membrane oxygenation (ECMO) underwent laparotomy. RESULTS: There were no significant differences in demographic characteristics between the two groups. Both nitric oxide (p = 0.13) and high-frequency oscillatory ventilation (p = 0.06) tended to be required more frequently in the laparotomy group than in the thoracoscopy group. A patch was applied to nine patients (five laparotomy, four thoracoscopy, p = 0.42). Mean operation time was longer for the thoracoscopy group, but not significantly so (p = 0.06). Times to feeding and lengths of hospital stays were similar, as were days of postoperative ventilator use and of total ventilator use. CDH recurred in two patients, both in the thoracoscopy group (p = 0.08), and intestinal obstruction occurred in five patients in the laparotomy group. Of eight patients who received ECMO treatment, only three survived. CONCLUSIONS: Except in patients receiving ECMO treatment, thoracoscopic repair is useful and feasible regardless of defect size or need for patching. Operation time is longer, but cosmetic appearance is better and intestinal obstruction uppers to be less frequent. However, intensive training is needed to prevent the recurrence.

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.

Neonatal neuroblastoma needs the aggressive treatment?

BACKGROUND: Routine antenatal ultrasound scans increased the detection of the neuroblastoma (NB) in neonates. We reviewed the treatment outcome and clinical presentation of neonatal NB. METHODS: We included patients who had pathologically confirmed NB presented within 28 days after birth from January 1999 to December 2010. RESULTS: There were 17 patients (8 females and 9 males), which consist of 16 % of total NB cases of children in our institution. Nine were followed from prenatal period as an abdominal mass and eight were presented postnatally (5 abdominal distensions, 2 tachypnea, and 1 persistent jaundice). The primary lesion was located in adrenal gland in ten patients, retroperitoneum in four, and posterior mediastinum in three. The tumor size was median 4.1 cm (range, 3-7). The stage of the patients were as follows: stage 1 in six, stage 2 in one, stage 3 in three, stage 4S in five, and stage 4 in two. Six patients were in the low-risk group, seven were intermediate-risk group, and four were high-risk group. Thirteen showed favorable histology among 15 specimens. Five patients (29.4 %) showed MYCN amplification. The median follow-up period was 78.4 months (range, 17.4-138.6). Fifteen of 17 (88.2 %) are alive without evidence of recurrences and two patients of stage 4S with MYCN amplification in high-risk group died. CONCLUSIONS: The overall survival of neonatal NB is 88.2 %, but we observed a high ratio of stage 4 and stage 4S tumors and MYCN amplification. We suggested that early treatment might be better for neonatal NB more than 3 cm in size. Aggressive treatment for neonatal NB could bring more favorable outcome.

Dissociation of diabetes and obesity in mice lacking orphan nuclear receptor small heterodimer partner.

Mixed background SHP(-/-) mice are resistant to diet-induced obesity due to increased energy expenditure caused by enhanced PGC-1α expression in brown adipocytes. However, congenic SHP(-/-) mice on the C57BL/6 background showed normal expression of PGC-1α and other genes involved in brown adipose tissue thermogenesis. Thus, we reinvestigated the impact of small heterodimer partner (SHP) deletion on diet-induced obesity and insulin resistance using congenic SHP(-/-) mice. Compared with their C57BL/6 wild-type counterparts, SHP(-/-) mice subjected to a 6 month challenge with a Western diet (WestD) were leaner but more glucose intolerant, showed hepatic insulin resistance despite decreased triglyceride accumulation and increased ß-oxidation, exhibited alterations in peripheral tissue uptake of dietary lipids, maintained a higher respiratory quotient, which did not decrease even after WestD feeding, and displayed islet dysfunction. Hepatic mRNA expression analysis revealed that many genes expressed higher in SHP(-/-) mice fed WestD were direct peroxisome proliferator-activated receptor alpha (PPARα) targets. Indeed, transient transfection and chromatin immunoprecipitation verified that SHP strongly repressed PPARα-mediated transactivation. SHP is a pivotal metabolic sensor controlling lipid homeostasis in response to an energy-laden diet through regulating PPARα-mediated transactivation. The resultant hepatic fatty acid oxidation enhancement and dietary fat redistribution protect the mice from diet-induced obesity and hepatic steatosis but accelerate development of type 2 diabetes.

Use of laparoscopic surgical resection for pediatric malignant solid tumors: a case series.

BACKGROUND: Minimally invasive surgery for malignant pediatric tumors remains controversial, and few cases have been published. The present study reports on our initial experiences of laparoscopic surgical resection for selected pediatric malignant solid tumors. METHODS: We retrospectively analyzed data from ten pediatric patients who underwent laparoscopic surgical resection for malignant solid tumors at our institute between April 2005 and January 2010. RESULTS: There were four boys and six girls, including one neonate and four infants. The mean age at surgery was 23.3 months (range, 13 days-69 months). Six patients underwent laparoscopic adrenalectomy for neuroblastoma (n=5) or adrenocortical carcinoma (n=1). Two patients underwent laparoscopic partial hepatectomy for hepatoblastoma, one patient underwent a laparoscopic salpingo-oophorectomy for yolk sac tumor, and one a laparoscopic tumor excision for rhabdomyosarcoma in the pelvis. Complete tumor resection was performed in all cases. Tumors ranged from 2.5 to 5.3 cm maximum diameter. Tumors were placed inside endobags and removed safely without spillage. The mean operation time was 132 (range, 65-250) min. There were no open conversions and no postoperative complications. The mean postoperative hospital stay was 4.9 (range, 2-7) days, and all surgical wounds showed good cosmetic results. There were no local tumor recurrences during the 17.3-month median follow-up period. CONCLUSIONS: Laparoscopic surgical resection for selected pediatric malignant solid tumors was found to be feasible and safe. Long-term follow-up data are essential to confirm oncologic safety.

Early vs. delayed surgery for choledochal cyst with acute pancreatitis in children.

BACKGROUND/AIMS: Few reports have appeared on the development of acute pancreatitis in children with choledochal cysts and no information on optimal timing of surgery is available. We assessed patient outcomes relative to timing of surgery and serum amylase and lipase concentrations. METHODOLOGY: We reviewed 29 patients < or = 15 years old with acute pancreatitis, as evidenced by abdominal pain, increased serum amylase and lipase concentrations (>200 IU/L for each), and radiological examination, who underwent surgery for congenital choledochal cysts between 2000 and 2010. RESULTS: Patients were divided into three groups according to timing of surgery. At the time of operation, serum amylase and lipase concentrations were significantly higher in patients who underwent early surgery. Change in hemoglobin concentration, length of operation, time of diet commencement after surgery, and hospitalization days, were similar in the three groups. Many patients for whom surgery was delayed underwent endoscopic retrograde cholangiopancreatography (ERCP). When patients were divided into three groups according to serum amylase and lipase concentrations at the time of operation, no statistically significant differences in surgical parameters or outcomes were evident. CONCLUSIONS: Early cyst excision following development of acute pancreatitis in patients with choledochal cysts reduces patient symptoms and decreases the need for ERCP.

Surgery for hepatoblastoma: from laparoscopic resection to liver transplantation.

BACKGROUND/AIMS: Introduction of liver transplantation and the application of minimally invasive surgery for selected hepatoblastoma patients made a tailored surgical approach possible according to the tumor status. METHODOLOGY: We retrospectively evaluated clinical outcomes of 38 pediatric patients with pathologically proven hepatoblastoma who underwent surgery at our institute between 1991 and 2009. Especially, we evaluated recent changes in clinical outcomes since the commencement of liver transplantations and laparoscopic resections for hepatoblastoma patients from 2007. RESULTS: Complete hepatic tumor resections including 5 liver transplantations were performed in all patients, with 5-year overall survival and event free survival rates of 74.8% and 73.8%, respectively. From 2007, we performed 5 liver transplantations for unresectable cases. No tumor recurrences occurred in any case after a 19.9 (14.5-35.6) months median follow-up period. Totally laparoscopic partial hepatectomies were performed (2 cases) for selected patients. Both cases showed early recovery without any complications and were free of disease recurrence after 8.1 and 19.3 months follow-up period. CONCLUSIONS: Though long term follow-up data is necessary for validation, we suggest that an individualized surgical strategy based on the accurate evaluation of the tumor extent might improve the clinical outcomes of patients with hepatoblastoma.

Effects of sirolimus in the treatment of unresectable infantile hemangioma and vascular malformations in children: A single-center experience.

OBJECTIVE: Recently, sirolimus has emerged as a safe and effective treatment modality for unresectable vascular lesions. In the present study, we investigated the effectiveness and safety of sirolimus from our early experience with patients with unresectable vascular anomalies. METHODS: The medical records and radiologic images of all patients with unresectable vascular anomalies treated with sirolimus at our center from January 2018 to November 2019 were retrospectively reviewed. All patients were administered oral doses of sirolimus 0.8 mg/m2 every 12 hours as the initial dose, followed by maintenance of a target serum concentration (5-10 ng/mL) with therapeutic drug monitoring. RESULTS: Six patients with unresectable vascular anomalies were treated with sirolimus for ≥10 months. Their median age at the initiation of sirolimus treatment was 17 months (range, 8-67 months). The median duration of treatment was 13 months (range, 10-16 months). One patient had a good response, four had an intermediate response, and one had no response to sirolimus therapy. None of the patients had discontinued sirolimus therapy because of adverse effects. CONCLUSIONS: Sirolimus can be used effectively and safely for patients with unresectable vascular anomalies. However, further prospective studies are warranted to evaluate the long-term effects of sirolimus and clarify the indications for early intervention.

The Advantages of Indocyanine Green Fluorescence Imaging in Detecting and Treating Pediatric Hepatoblastoma: A Preliminary Experience.

Background: Currently, indocyanine green (ICG) fluorescence imaging enables radical surgical resection in hepatoblastoma (HB) and has beneficial uses; however, its usage in pediatric patients is still limited. Methods: From 2015 to 2019, 17 hepatoblastoma patients underwent 22 fluorescence-guided surgery using ICG. ICG (0.3 mg/kg) was intravenously injected 24-48 h before the operation. With ICG/NIR camera, intraoperative identification of biological structures and demarcation of mass were conducted. Results: ICG fluorescence-guided surgery was performed for hepatoblastoma in 22 cases: 16, 1, and 2 cases underwent anatomic resection, partial hepatectomy, and liver transplantation, respectively. Six patients accompanied lung metastasis at the time of surgery, and two patients underwent lung surgery using ICG. The median interval from ICG injection to surgery was 38.3 h (range, 20.5-50.3 h). The median tumor size was 36.5 mm (range, 2-132 mm). According to the pathologic finding, the median safety margin was secured for 6 mm (range, 0-11 mm) and there was no residual finding at the liver at the follow-up computed tomography (CT). Conclusions: ICG fluorescence imaging in children with HB was feasible and safe for tumor demarcation and enhancing the accuracy of radical tumor resection.