Quantitative evaluation of pediatric umbilical loop stomas: 2 decades of experience from a single institution.

PURPOSE: Since pediatric stomas are often temporary, their creation, management, and closure should be simple, with minimal complications and excellent cosmetic results. We began employing umbilical stomas in 2000. This study aimed to characterize the ingenuity and utility of umbilical stomas and provide a quantitative evaluation of their cosmetic outcomes. METHODS: We examined cases of stoma construction and closure surgery performed in our department from January 2000 to December 2022. The umbilical and non-umbilical stoma groups included 54 and 42 cases, respectively, and the findings for both groups were compared and analyzed. RESULTS: The two groups showed no significant differences in the incidence of complications. The Manchester Scar Scale score for the umbilical stoma group (8.42 ± 1.85) was significantly better than that for the non-umbilical stoma group (16.31 ± 2.96; P < 0.01). Likewise, in Patient and Observer Scar Assessment Scale assessments, the umbilical stoma group showed significantly better scores in both the observer scale (9.48 ± 2.50 vs. 21.78 ± 7.26; P < 0.01) and the patient scale (10.5 ± 1.39 vs. 22.40 ± 7.35; P < 0.01). CONCLUSIONS: Umbilical stomas are easy to manage and yield an inconspicuous closure incision with excellent cosmetic outcomes. Although patient selection is important, pediatric umbilical stomas are a valuable option that can be actively employed.

Preferential performance of Thal fundoplication for gastroesophageal reflux disease: a single institution experience.

PURPOSE: Nissen fundoplication (NF) is the most commonly used surgical treatment for persistent gastroesophageal reflux disease (GERD). We introduced to the alternative Thal fundoplication (TF) (partial anterior wrapping) in 1998. The purpose of this paper is to review and report on the effectiveness of TF in our department. METHODS: We retrospectively analyzed cases of 281 patients who underwent TF for GERD at our hospital from 1998 to 2019. RESULTS: Average age, 16.3 ± 18.1 years; average body weight, 21.0 ± 16.0 kg; average operative time, 89.1 ± 43.0 min; average volume of bleeding, 11.6 ± 29.2 g; enteral feeding commenced after an average of 3.4 ± 1.3 postoperative days (PODs), and average postoperative full enteral feeding was 6.3 ± 1.4 PODs. Five patients (1.8%) had Clavien-Dindo classification III or higher; average hospital stay duration was 10.3 ± 6.0 days, with symptom recurrence affecting 17 patients (6.1%). CONCLUSION: TF may be an effective and simple treatment for GERD that has few recurrences and avoids complications common to NF, but further studies to compare it with other techniques are needed.

Risk factors for recurrent gastroesophageal reflux disease after Thal fundoplication.

PURPOSE: The recurrence rate of gastroesophageal reflux disease (GERD) after fundoplication has been reported to be 7-25%. We investigated the risk factors for recurrence of GERD after Thal fundoplication (TF) in our department with the aim of further reducing the recurrence rate of GERD. METHODS: We retrospectively analyzed 276 patients who underwent TF for GERD at our hospital between 2000 and 2019. Retrospectively considered variables were obtained from the medical records of patients. The variables included patient characteristics, GERD severity, surgery-related factors and postoperative course. RESULTS: The postoperative GERD recurrence rate was 5.8%. In the univariate analysis, the presence of convulsive seizures (12/4 vs. 110/150, p = 0.046) and the absence of a tracheostomy (0/16 vs. 53/207, p = 0.048) at the time of TF were significantly associated with recurrence. In the multivariate analysis, the presence of convulsive seizures at the time of TF was the only factor significantly associated with recurrence. CONCLUSION: The presence of convulsive seizures and the absence of a tracheostomy at the time of TF were significantly associated with GERD recurrence after TF. Active control of seizures and consideration of surgical indications, including assessment of respiratory status, are important in preventing the recurrence of GERD after TF.

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 an external lengthening procedure on the outcome of long-gap esophageal atresia at our hospitals.

PURPOSE: To demonstrate the outcome of external lengthening for long-gap esophageal atresia (LGEA) at our hospitals. METHODS: Five patients with LGEA underwent external lengthening between 2010 and 2014 (group A), and 11 patients with LGEA underwent other lengthening techniques between 1990 and 2011 (group B). We compared the procedure and outcome between these two groups. RESULTS: The mean birth weight was 2001 g in group A and 2485 g in group B (p = 0.06). The mean age at esophageal reconstruction was 28 days in group A and 227 days in group B (p = 0.03). Although primary esophageal anastomosis without myotomy was feasible in all patients in group A, a myotomy was needed for primary esophageal anastomosis in half of the patients in group B. Anastomotic leakage occurred in none in group A and in six patients in group B (p = 0.03). The mean age at the establishment of full oral feeding was 76 days in group A and 686 days in group B (p = 0.009). CONCLUSION: External traction for LGEA can effectively lengthen the esophagus to enable primary anastomosis at an earlier age. This may facilitate oral intake.

Navigation using indocyanine green fluorescence imaging for hepatoblastoma pulmonary metastases surgery.

To achieve precise and sensitive detection of chemotherapy-resistant hepatoblastoma pulmonary metastases, we performed surgery using indocyanine green (ICG) fluorescence imaging navigation. Lung metastasectomies were performed in 10 patients aged from 1 to 11 years. ICG (0.5 mg/kg) was injected intravenously 24 h before the operation. After a thoracotomy had been performed, a 760-nm infrared ray was applied to the lung using a generator and the 830-nm evoked fluorescence was collected and visualized on a real-time display. In total, 250 fluorescence-positive lesions were extirpated in 37 operations. All of the pathologically positive lesions were clearly fluorescence positive. The diameter of the smallest detectable lesion was 0.062 mm. In two patients, there were 29 extirpated lesions that were pathologically proven not to be hepatoblastoma metastases. Although a problem of false positive remains, this method is very useful for the detection of small pulmonary metastases.

Type IV laryngotracheoesophageal cleft repair by a new combination of lateral thoraco-cervical and laryngoscopic approaches.

Type IV laryngotracheoesophageal cleft (LTEC) is a rare congenital anomaly that is associated with high morbidity and mortality despite various forms of surgical repair. This article presents our strategy for surgical management of type IV LTECs using a combination of lateral thoraco-cervical and laryngoscopic approaches.

A case series of prophylactic negative pressure wound therapy use with purse-string closure in stoma closure wounds in infants.

BACKGROUND: The study introduces the application of negative pressure wound therapy (NPWT) in pediatric stoma closure, emphasizing the importance of enhancing aesthetics and minimizing surgical site infections (SSI). CASE PRESENTATION: The case series involves four infants undergoing non-umbilical stoma closure with a combination of purse-string closure (PSC) and NPWT, focusing on aesthetic outcomes and infection prevention. NPWT was initiated immediately after surgery, and patients were monitored every 3-4 days. Notably, none of the four infants experienced SSI or other complications. The patients adequately tolerated NPWT, with no significant adverse events. Furthermore, Manchester Scar Scale (MSS) was 9 [7-10], and Patient and Observer Scar Assessment Scale (POSAS) (observer) was 12.5 [12-19], POSAS (patient) was 12.5 [11-16] (all median values [minimum-maximum]), indicating that excellent aesthetic outcomes were achieved. DISCUSSION: We emphasizes the significance of aesthetics in pediatric patients; in addition, our findings demonstrate that four infants who received NPWT combined with PSC achieved superior outcomes that did the most recent four infants who underwent PSC only at our institution. It also addresses the risk of SSI in stoma closure and discusses the pros and potential cons of using NPWT in pediatric cases, underlining the need for further research and the accumulation of additional reports. CONCLUSIONS: This is the inaugural report of prophylactic NPWT for pediatric stoma closure, emphasizing the effectiveness of combining PSC and NPWT for SSI prevention and improved aesthetics. The study calls for additional research and reports on NPWT in pediatric cases to further solidify its benefits in this patient population.

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.

Case of laparoscopic-assisted anorectoplasty performed with temporary umbilical loop colostomy for high anorectal malformation (rectovesical fistula): a three-stage minimally invasive surgery.

This is the first report of three-stage laparoscopic-assisted anorectoplasty (LAARP) with temporary umbilical loop colostomy aiming for minimally invasive surgery in a boy with high anorectal malformation. The procedure was performed safely and resulted in small inconspicuous wounds. LAARP with temporary umbilical loop colostomy was a sufficiently useful therapeutic approach to high anorectal malformation.

Advantage of Using 8K Ultra-High-Definition Television System for Kasai Portoenterostomy for Biliary Atresia.

Kasai portoenterostomy (KPE) is currently the first-line treatment for biliary atresia. Many pediatric surgeons have reported that the dissection of the fibrous remnant at the porta hepatis is one of the most important components of this procedure. Furthermore, laparoscopic portoenterostomy is being increasingly used to treat biliary atresia. An advantage of laparoscopic surgery is that surgeons can more easily identify microbiliary ducts, owing to the magnification. We report the case of a 61-day-old girl on whom we performed an exploratory laparotomy and diagnosed type III biliary atresia using intraoperative cholangiography. For the first time, we performed an open KPE using an 8K ultra-high-definition television system. This allowed us to clearly view the porta hepatis and to successfully perform the portoenterostomy.

Damage control surgery for grade IV blunt hepatic injury with multiple organ damage in a child: a case report.

BACKGROUND: Intra-abdominal hemorrhage caused by blunt hepatic injury is a major cause of morbidity and mortality in patients with abdominal trauma. Some of these patients require laparotomy, and rapid decision-making and life-saving surgery are essential. Damage control (DC) surgery is useful for treating children in critical situations. We performed this technique to treat an 8-year-old boy with grade IV blunt hepatic injury and multiple organ damage. This is the first report of the use of the ABTHERA Open Abdomen Negative Pressure Therapy System (KCI, now part of 3 M Company, San Antonio, TX, USA) for DC surgery to rescue a patient without neurological sequelae. CASE PRESENTATION: An 8-year-old boy was brought to the emergency department of our hospital after being run over by a motor vehicle. He had grade IV blunt hepatic injury, thyroid injury, and bilateral hemopneumothorax. Although he was hemodynamically stable, the patient's altered level of consciousness, the presence of a sign of peritoneal irritation, and suspicion of intestinal injury led us to perform exploratory laparotomy. As part of a DC strategy, we performed gauze packing to control hemorrhage from the liver and covered the abdomen with an ABTHERA Open Abdomen Negative Pressure Therapy System to improve the patient's general condition. Eighteen days after admission, the patient was diagnosed with a biliary fistula, which improved with percutaneous and external drainage. He had no neurological sequelae and was discharged 102 days after injury. CONCLUSION: The DC strategy was effective in children with severe blunt hepatic injury. We opted to perform DC surgery because children have less hemodynamic reserve than adults, and we believe that using this strategy before the appearance of trauma triad of death could save lives and improve outcomes. During conservative management, it is important to adopt a multistage, flexible approach to achieve a good outcome.

Menkes disease with gastroesophageal reflux disease and successful surgical treatment: a case report and literature review.

The complication of Menkes disease (MD) and gastroesophageal reflux disease (GERD) is extremely rare. This report describes the very rare case of a one-year-old boy with MD complicated with GERD, and the successful surgical treatment of GERD. We review the literature on this relationship between MD and GERD, and discuss the clinical features of this association. Furthermore, the possible cause of the onset of GERD complicated with MD is explored in this report.

Congenital multiple colonic atresias with intestinal malrotation: a case report.

BACKGROUND: Congenital intestinal atresia develops in 1 in 1500 to 20,000 births. Colonic atresia, which accounts for 1.8-15% of intestinal atresia cases, is accompanied by other gastrointestinal atresias such as small intestinal atresia, gastroschisis, imperforate anus, and intestinal malformation in 47-80%. Although a report shows that patients with multiple colonic atresias are 8.9% of those with colonic atresia. CASE PRESENTATION: A male infant did not have the first bowel movement within 36 h of birth and had abdominal distention/vomiting. Radiography showed significant dilation of the intestinal tract. A contrast enema examination at 3 days of age showed a microcolon and disruption in the descending colon. We performed an emergency decompressive loop enterostomy in the distended segment. At the age of 7 months, imaging from the stoma showed disruption of the contrast medium in the intestinal tract at the right lower abdomen, and the continuity of the intestinal tract was not clarified. Intestinal malrotation was found during the second surgery, and the enterostomy was located in the ileum proximal to Bauhin's valve. Continuity of the intestinal serosal surface was maintained. However, multiple membranous obstructions (three atresias and one stenosis) were observed in the distal segment of the bowel, which was penetrated by intraluminal advancement of a urethral catheter. Therefore, he was diagnosed with multiple colonic atresias. The intestinal tract was longitudinally incised, and membranectomy and mucosal/lateral suture were performed. CONCLUSIONS: It is important for neonates with intestinal atresia to evaluate and prepare for distal patency of the colon before radical anastomosis. In addition, anomalies associated with colon atresia should also be assessed.

Spontaneous pneumothorax caused by an inflammatory myofibroblastic tumor-like lesion in a 14-year-old girl: a case report.

BACKGROUND: Spontaneous pneumothorax occurs more often in younger, slim, and shallow-chested men. Although less common, differential diagnoses for secondary pneumothorax in children are asthma, emphysematous blebs, catamenial pneumothorax, and others. We report a patient who presented with pneumothorax and was found to have an inflammatory myofibroblastic tumor (IMT)-like lesion, and present a review of the related literature. CASE PRESENTATION: A 14-year-old girl visited her physician for chest pain that developed while exercising. Although chest drainage was performed, the symptoms associated with a collapsed lung did not improve, and she was referred to our hospital. Computed tomography revealed the presence of a 19 × 17-mm cyst with a thick wall in the apex of the right lung. She was tested for infectious diseases, namely tuberculosis, but the results were not definitive. Catamenial pneumothorax was also suspected because she was menstruating when she presented to our hospital. As a therapeutic diagnosis, we performed a thoracoscopic partial resection of the right upper lobe of the lung. Three small openings were identified inside the cyst, suggesting connection with the bronchiole. The lesion was pathologically diagnosed as an IMT-like lesion. Considering the progress so far, we considered that the final diagnosis to be an IMT. The patient was discharged on postoperative day 3, and we have followed her for the past 6 months with no local recurrence or metastasis. CONCLUSIONS: IMT is not uncommon in children. Therefore, this lesion should be considered as a possible diagnosis if children and young adults develop spontaneous pneumothorax.

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.

[A case of long-term survival of unresectable-advanced gastric cancer due to chemotherapy].

The case was a 50s male with chief complaints of body weight loss and nausea. A clinical finding was Stage IV gastric cancer of poorly differentiated adenocarcinoma. We diagnosed it unresectable and started 5-FU+CDDP as the first-line chemotherapy. Partial response (PR) was observed and progression free time was 7 months. After 9 courses of 5-FU+ CDDP, the tumor grew and an oral intake was getting impossible. Gastro-jejunostomy was performed and then started a weekly PTX as the second-line chemotherapy after operation. The response was progressive disease (PD) after 4 courses of weekly PTX. Then we started S-1+CPT-11 as the third-line chemotherapy. We could continue S-1+CPT-11 for 9 courses without a severe adverse effect. Overall survival was 26.2 months.