Transumbilical Repair for Umbilical Hernia: A New Technique.

Although there are many reports on surgical repair for umbilical hernia, there is no standard procedure at present. Since 2012, we have performed surgery with transumbilical repair using an original procedure. With this procedure, a longitudinal incision is made in the umbilicus, and the fascial defect is closed. Excess skin is excised at a fixed length. The fascia and dermis are sutured vertically over a length of 15 mm. A total of 424 patients with pediatric umbilical hernia who underwent this procedure between September 2012 and December 2020 were reviewed. The mean operative duration was 52 minutes. All patients were followed up to 6 months after surgery. Postoperative complications included infection in 15 patients and wound granulation in 5 patients. The morphology of the umbilicus is natural and satisfying. We conclude that this procedure is safe and simple and the results are satisfactory.

A novel risk stratification model based on the Children's Hepatic Tumours International Collaboration-Hepatoblastoma Stratification and deoxyribonucleic acid methylation analysis for hepatoblastoma.

INTRODUCTION: Hepatoblastoma (HB) is the most common paediatric liver tumour, and epigenetic aberrations may be important in HB development. Recently, the Children's Hepatic Tumors International Collaboration-Hepatoblastoma Stratification (CHIC-HS) developed risk stratification based on clinicopathological factors. This study aimed to construct a more accurate model by integrating CHIC-HS with molecular factors based on DNA methylation. METHODS: HB tumour specimens (N = 132) from patients treated with the Japanese Pediatric Liver Tumors Group-2 protocol were collected and subjected to methylation analysis by bisulfite pyrosequencing. Associations between methylation status and clinicopathological factors, overall survival (OS), and event-free survival (EFS) were retrospectively analysed. We investigated the effectiveness of the evaluation of methylation status in each CHIC-HS risk group and generated a new risk stratification model. RESULTS: Most specimens (82%) were from post-chemotherapy tissue. Hypermethylation in ≥2 of the four genes (RASSF1A, PARP6, OCIAD2, and MST1R) was significantly associated with poorer OS and EFS. Multivariate analysis indicated that ≥2 methylated genes was an independent prognostic factor (hazard ratios of 6.014 and 3.684 for OS and EFS, respectively). Two or more methylated genes was also associated with poorer OS in the CHIC-very low (VL)-/low (L)-risk and CHIC-intermediate (I) risk groups (3-year OS rates were 83% vs. 98% and 50% vs. 95%, respectively). The 3-year OS rates of the VL/L, I, and high-risk groups in the new stratification model were 98%, 90%, and 62% (vs. CHIC-HS [96%, 82%, and 65%, respectively]), optimising CHIC-HS. CONCLUSIONS: Our proposed stratification system considers individual risk in HB and may improve patient clinical management.

A MicroRNA Cluster in the DLK1-DIO3 Imprinted Region on Chromosome 14q32.2 Is Dysregulated in Metastatic Hepatoblastomas.

Hepatoblastoma (HB) is the most common malignant liver neoplasm in children. Despite progress in HB therapy, outcomes for patients with metastatic disease remain poor. Dysregulation of miRNA expression is one of the potential epigenetic mechanisms associated with pathogenesis of HB. However, miRNA profiles related to the different stages of HB tissues and cells, in particular of lung metastatic tumor cells, are unknown. In the present study, using array-based miRNA expression and DNA methylation analysis on formalin-fixed paraffin-embedded tissues, we aimed to identify miRNA changes that can discriminate between lung metastatic tumors, primary tumors (fetal and embryonal subtypes), and nontumorous surrounding livers. Our analysis demonstrated that a large cluster of microRNAs and snoRNAs located within the 14q32.2 DLK1-DIO3 region showed a strikingly upregulated expression pattern in HB tumors, especially metastatic tumors, compared to normal liver tissues. This revealed dysregulation of miRNAs similar to that seen in a malignant stem-like subtype of hepatocellular carcinoma associated with poor prognosis. These findings in HB mirror similar findings made in multiple other cancer types. With further analysis this may in future allow stratification of different stages and types of HB tumors based on their miRNA profiles, which could lead to new approaches to diagnosis and treatment in progressive HB patients.

Hypermethylation of CSF3R is a novel cisplatin resistance marker and predictor of response to postoperative chemotherapy in hepatoblastoma.

AIM: Most hepatoblastoma patients undergo pre/postoperative cisplatin treatment. Approximately 20% patients are cisplatin resistant, and show poor prognosis and high recurrence rates. However, some cisplatin-sensitive patients show early recurrence. We consider that a small population of cisplatin-resistant cells may remain after preoperative chemotherapy. Previous studies showed a correlation between DNA hypermethylation and hepatoblastoma progression. Here, we examined whether DNA hypermethylation was related to cisplatin resistance and could be a potential indicator for cisplatin as postoperative chemotherapy. METHODS: We extracted DNA from 43 resected hepatoblastoma tumors. Methylation array analyses were performed in 11 samples, including six cisplatin-sensitive and five cisplatin-resistant samples. We also performed cDNA microarray analysis in parental and cisplatin-resistant HuH6 cells. Through comparison of the datasets, we selected the strongest correlated cisplatin-resistant candidate gene. Using bisulfite pyrosequencing, the candidate gene methylation level was assessed in 38 cisplatin-sensitive patients after checking its usefulness as a substitute modality of methylation array. Correlations between the methylation status and clinical data were analyzed. RESULTS: CSF3R was the strongest correlated variable. Bisulfite pyrosequencing analysis also confirmed CSF3R was significantly hypermethylated in cisplatin-resistant patients. Among the 38 cisplatin-sensitive patients, recurrence curves showed that the CSF3R high methylation patients had significantly higher recurrence than CSF3R low methylation patients. The recurrence curve of methylation high patients was similar to that of cisplatin-resistant patients. CONCLUSIONS: Our findings suggested that CSF3R hypermethylation was related to cisplatin resistance in HB patients and could be a predictor of postoperative chemotherapy, and indicate that CSF3R high methylation patients should be treated with non-CDDP regimens.

One-Stage Laparoscopic Surgery for Pulmonary Sequestration and Hiatal Hernia in a 2-Year-Old Girl.

We herein report a case of one-stage laparoscopic surgery for extralobar pulmonary sequestration (EPS) and hiatal hernia. Our patient was a 2-year-old girl who was diagnosed as a mediastinal mass lesion. Postnatal computed tomography revealed that the mediastinal mass was an EPS. Two weeks after birth, the patient developed gastroesophageal reflux (GER), and esophagography showed a hiatal hernia. At 2 years of age, she underwent one-stage laparoscopic Nissen's fundoplication for GER with resection of the EPS in the posterior mediastinum. The sequestrated lung was grasped via the esophageal hiatus; three aberrant blood vessels were dissected to allow removal of the sequestration through the umbilical port site. The esophageal hiatus was repaired and Nissen's fundoplication was performed laparoscopically. The patient's postoperative course was uneventful, with no recurrence of GER symptoms for 1 year. We conclude that one-stage laparoscopic surgery is useful for patients with EPS and hiatal hernia.

Differential diagnosis of a large size tumor in the retroperitoneum: A case report of retroperitoneal lipoblastoma.

Lipoblastoma can arise almost anywhere within the soft tissues, especially in the extremities. It is rarely seen retroperitoneally. A 3-year-old girl presented with a palpable abdominal mass underwent an exploratory laparotomy with resection of the retroperitoneal mass. Based on histopathologic and cytogenetic features, a final diagnosis of lipoblastoma was rendered. At 18-month follow-up, she had no evidence of recurrence.

A staged operation for anopenile urethral fistula.

Anopenile urethral fistula (APUF) is not rare, but there are only a few detailed reports regarding its surgical treatment. We describe a patient with APUF who had no anal opening, and meconium was discharged from the external urethral orifice. The patient was treated with a staged operation.

Clinical prognostic value of DNA methylation in hepatoblastoma: Four novel tumor suppressor candidates.

Hepatoblastoma (HB) is very rare but the most common malignant neoplasm of the liver occurring in children. Despite improvements in therapy, outcomes for patients with advanced HB that is refractory to standard preoperative chemotherapy remain unsatisfactory. To improve the survival rate among this group, identification of novel prognostic markers and therapeutic targets is needed. We have previously reported that altered DNA methylation patterns are of biological and clinical importance in HB. In the present study, using genome-wide methylation analysis and bisulfite pyrosequencing with specimens from HB tumors, we detected nine methylated genes. We then focused on four of those genes, GPR180, MST1R, OCIAD2, and PARP6, because they likely encode tumor suppressors and their increase of methylation was associated with a poor prognosis. The methylation status of the four genes was also associated with age at diagnosis, and significant association with the presence of metastatic tumors was seen in three of the four genes. Multivariate analysis revealed that the presence of metastatic tumors and increase of methylation of GPR180 were independent prognostic factors affecting event-free survival. These findings indicate that the four novel tumor suppressor candidates are potentially useful molecular markers predictive of a poor outcome in HB patients, which may serve as the basis for improved therapeutic strategies when clinical trials are carried out.

Impact of umbilical polyp resection: A report and literature review.

The preferred treatment for this lesion should be the total resection of the umbilical polyp with a mini-laparotomy because residual intestinal mucosa at the umbilicus can be confirmed to eliminate the risk of recurrence.

High drain amylase and lipase values predict post-operative pancreatitis for choledochal cyst.

BACKGROUND: Post-operative pancreatitis is a severe complication after cyst excision with hepaticoenterostomy (CEHE) for choledochal cysts. The aim of this study was to examine the dynamic post-operative changes in drain amylase and lipase values after CEHE for choledochal cysts, and then compare these values with the clinical outcomes in order to identify risk factors for post-operative pancreatitis after CEHE. PATIENTS AND METHODS: A total of 19 patients with choledochal cysts were retrospectively examined in the period between 2005 and 2012. The amylase and lipase values in the drainage and the serum, and the output of the effluent were measured post-operatively. The associations between their values and the clinical outcomes were evaluated. RESULTS: Six were found to have a pancreatic leak according to an international study group definition. In two of them, who developed post-operative pancreatitis, both amylase and lipase values in drainage were markedly elevated at 1 post-operative day (1 POD). The drain amylase value seemed to elevate rather specifically dependent on the occurrence of post-operative pancreatitis, whereas the drain lipase value tended to elevate regardless of the presence/absence of post-operative pancreatitis. CONCLUSION: It was indicated that amylase and lipase values in drainage at 1 POD could be effective predictors of post-operative pancreatitis after CEHE.

RASSF1A methylation indicates a poor prognosis in hepatoblastoma patients.

PURPOSE: The RAS association domain family protein 1 (RASSF1A) is known to be frequently inactivated by promoter hypermethylation in cancers. This study investigated the association of RASSF1A methylation with clinical outcomes in hepatoblastoma patients and whether it is correlated with the histological phenotype of hepatoblastoma tumors. METHODS: Seventy-four hepatoblastoma tumors were obtained from patients enrolled in the Japanese study group for pediatric liver tumor protocol-2. From nine formalin-fixed, paraffin-embedded specimens, we extracted DNA by dissection under a light microscope. We examined the methylation status of the RASSF1A promoter region by bisulfite pyrosequencing. RESULTS: Twenty-five (33.8 %) hepatoblastoma tumors were classified as having methylated RASSF1A. The RASSF1A methylation was significantly associated with metastatic tumors and a poor prognosis. Despite the complete resection, five pretreatment extent of disease II tumors showed recurrence or distant metastasis postoperatively. Among these cases, four tumors were found to show RASSF1A methylation. When compared to histologically different types of cell, RASSF1A methylation values in samples of the normal liver, fetal type, and embryonal type, were significantly elevated in ascending order. CONCLUSIONS: We confirmed that RASSF1A methylation is a significant prognostic indicator in hepatoblastomas, and it may become a promising molecular marker to stratify patients into appropriate risk groups.

Hemorrhagic shock due to spontaneous rupture of adrenal neuroblastoma in an infant: a rare case and review of the literature.

Spontaneous rupture of adrenal neuroblastoma is very rare in infants, in contrast to neonates. This report describes a 9-month-old boy presenting with acute hemorrhagic shock due to spontaneous rupture of adrenal neuroblastoma. MYCN oncogene amplification may be a predisposing factor for spontaneous rupture and bleeding of neuroblastoma. An appropriate surgical treatment for this condition must be discussed according to the patient's general state and the tumor features, such as staging, the origin, and local invasiveness.

Meconium pseudocyst with particular pathologic findings: a case report and review of the literature.

Meconium peritonitis is a sterile chemical peritonitis caused by bowel perforation with intraperitoneal extravasation of the meconium in utero. When the inflamed intestinal loops become fixed, meconium peritonitis leads to a cystic cavity with a fibrous wall, and the result is termed cystic-type meconium peritonitis. On the contrary, a meconium pseudocyst has a muscle layer continuous with the normal intestine and is distinguished from cystic-type meconium peritonitis based on the histopathologic findings. This report describes the rare case of a neonate complicated by a meconium pseudocyst, which was successfully treated with 1-stage resection and primary anastomosis. There have been few cases of meconium pseudocysts reported in the literature. Meconium peritonitis should be considered in the differential diagnosis in patients who develop large abdominal cysts with air and fluid content. Cystic-type meconium peritonitis is usually treated using drainage with subsequent elective surgery. However, for a meconium pseudocyst, 1-stage intestinal resection with primary anastomosis may be recommended. A meconium pseudocyst may be treatable using 1-stage resection based on histopathologic features.

Chylous ascites caused by resection of a choledochal cyst.

Chylous ascites is a rare complication of abdominal surgery in children. Particularly, reports of postoperative chylous ascites are rare. This report describes the very rare case of a 10-month-old girl complicated by chylous ascites after resection of a choledochal cyst with a Roux-en-Y hepaticojejunostomy, who was successfully treated medically. To date, we have found a few cases of postoperative chylous ascites in the paediatric literature. To the best of our knowledge, this is the first report of chylous ascites after the resection of a choledochal cyst in a child who was successfully treated solely by no fasting. No fasting might be a therapeutic option of paediatric postoperative chylous ascites after the resection of a choledochal cyst if the outflow volume of chylous ascites is small.

Hepatic osteodystrophy complicated with bone fracture in early infants with biliary atresia.

Biliary atresia (BA) is one of the major hepatobiliary abnormalities in infants and one of the causes of hepatic osteodystrophy. Bone disease may be caused by the malabsorption of calcium and magnesium by vitamin D in hepatobiliary diseases in which bile flow into the intestines is deficient or absent. Bone fracture before Kasai hepatic portoenterostomy or within one month after the procedure in an infant with BA is very rare. We herein report two infants: one infant with BA who initially presented with a bone fracture before Kasai hepatic portoenterostomy, and the other at 4 wk after Kasai hepatic portoenterostomy, and also provide a review of the literature. Moreover, we conclude that clinicians should consider BA in infants with bone fracture during early infancy.

Nissen fundoplication for gastroesophageal reflux: No deterioration of gastric emptying measured by C-acetate breath test.

AIM: To study the gastric emptying 30 days after laparoscopic Nissen fundoplication (NF) in gastroesophageal reflux. MATERIALS AND METHODS: Three patients were evaluated with (13)C-acetate breath test (ABT) performed pre and post-NF. The liquid test meal consisted of Racol(™) mixed with (13)C-acetate. RESULTS: In the patient without neurological impairment (NI), the preoperative t(½)ex and t lag were 0.900 and 0.510 hours, respectively. The postoperative t(½)ex and t lag were 0.959 and 0.586 hours, respectively. In one patient with NI, the preoperative t(½)ex and t lag were 1.828 and 1.092 hours, respectively. The postoperative t(½)ex and t lag were 2.081 and 1.025 hours, respectively. In the other patient with NI, the preoperative t(½)ex and t lag were 2.110 and 0.980 hours, respectively. The postoperative t(½)ex and t lag were 1.118 and 0.415 hours, respectively. CONCLUSIONS: Our findings suggest that (13)C-ABT parameters did not worsen in any of the children after laparoscopic NF.

Acoustic emission measurement using a strain-insensitive fiber Bragg grating sensor under varying load conditions.

Acoustic emission (AE) during a pressure test of a carbon fiber-reinforced plastic tank was measured using a fiber Bragg grating (FBG) sensor and, for comparison, using a conventional piezoelectric sensor. The FBG-inscribed optical fiber was used not only as an optical transmission line but also as an ultrasonic transmission line. The FBG sensor showed resonant characteristics and could detect AE continuously during the test. AE detected with the FBG sensor exhibited a cumulative behavior similar to that detected with the piezoelectric sensor. The FBG sensor was demonstrated to have comparable AE detection capability to piezoelectric sensors.

Successful treatment of intravascular large B-cell lymphoma diagnosed by bone marrow biopsy and FDG-PET scan.

Early diagnosis of intravascular large B-cell lymphoma (IVLBCL) is difficult, but is critical for longer survival for the patients. We report a case of IVLBCL that was diagnosed with the help of FDG-PET. A 76-year-old woman was referred to us for the evaluation of her elevated serum LDH. She presented with general malaise and high fever. There were no skin lesions or neurological involvement. FDG-PET imaging showed increased uptake of FDG in the vertebra, bilateral femurs, sternum, and iliac bones. A diagnosis of IVLBCL was made by bone marrow biopsy. She was successfully treated with rituximab and modified CHOP therapy.