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Purpose: To assess the long-term cosmetic outcomes of the "slit-slide procedure", designed to provide a more natural appearance for umbilical hernia repair, as perceived by the patients and their parents. Methods: A total of 149 patients with umbilical hernia underwent the slit-slide procedure at our hospital. The slit-slide procedure allows for the creation of an umbilicus with a more natural and integrated appearance. The patient satisfaction survey questionnaire was mailed to the families (n = 139), and there were 74 (53.2%) respondents. A questionnaire survey on postoperative appearance was also distributed to pediatric surgeons. Results: The median age at the time of operation was 2.5 years (range, 2 months to 14 years) and the average median age at the time of answering the questionnaire was 6.25 years old (range, 2.5–14.8 years). The average median period of observation was 3.2 years (range, 4 months to 8.97 years). Most patients (89.2%) and parents (93.2%) were satisfied with the appearance of the umbilicus. Twenty-seven patients reported improved satisfaction after surgery (36.2%). Surgeons tended to score the elongated-oval shape highly; however, there was no difference in satisfaction among the shapes. Conclusion: The slit-slide procedure is not only effective and safe, but it achieves a satisfactory aesthetic outcome. [ABSTRACT FROM AUTHOR]

Abstract: Objectives: The reserve of the venous route to the central veins is important for long-term parenteral nutrition (PN). Frequent catheter-related bloodstream infection (CRBSI) induces occlusion of the venous routes. Therefore, a modified exchange procedure using a tunneled central venous catheter (CVC) with a fibrous sheath was developed to preserve the route to the central veins. Methods: Seven patients who required long-term PN received the modified exchange procedure and the outcome of exchanged CVC was retrospectively reviewed. Results: The procedure was performed 10 times in seven patients. The venous routes were either the subclavicular or the internal jugular vein in all patients. The exchange of the catheter was due to CRBSI or occlusion in almost all patients. The mean duration of new catheter use was 296.2 days following the exchange. Four catheters continued to be used, and the remaining ones were removed. The reasons for removal were severe CRBSI and occlusion, each of which occurred in two catheterized patients, while the reason for removing the remaining catheters was because the patients no longer needed the catheters. Conclusion: The modified catheter exchange using fibrous sheath, even in patients with CRBSI, appears to be an effective procedure for reserving the venous route to the central veins in patients who require either long-term PN or other treatments. [Copyright &y& Elsevier]

Purpose: Both the mortality and morbidity associated with congenital diaphragmatic hernia (CDH) are mainly caused by pulmonary hypoplasia and persistent pulmonary hypertension. A previous study revealed that insulin-like growth factors (IGFs) play important roles in fetal lung development. The aim of this study was to investigate the effect of IGF-1 and IGF-2 on tissue cultures of fetal hypoplastic lungs obtained from nitrofen-induced CDH model rats.Methods: Pregnant rats were exposed to nitrofen on day 9 of gestation (D9). Fetuses were harvested on D18 by caesarian section. Lung specimens of the CDH (+) fetus were divided into three groups; control, IGF-1, and IGF-2. The specimens from the control group were cultured in culture medium without IGFs. The IGF-1 group specimens were cultured with IGF-1 (500 ng/ml), and those in the IGF-2 group were cultured with IGF-2 (500 ng/ml). The mRNA expression of TTF-1, T1α and α-SMA were analyzed in each group using real-time RT-PCR after 24 and 48 h of incubation. Immunohistochemical staining of these markers was also assessed for each of the cultured specimens.Results: There was a significant increase in the expression of both TTF-1 and T1α mRNA in the IGF-2 group, in comparison to the control group after 48 h of culture. Immunohistochemical staining revealed that the cell morphology was changed from cuboidal to squamous type in the IGF-2 group.Conclusions: An increased mRNA expression of the markers related to type 1 and 2 alveolar epithelial cells, and morphological changes in the epithelial cells were observed in the IGF-2 group. The administration of IGF-2 to nitrofen-induced hypoplastic lungs might lead to alveolar maturation, which thus results in their improved development. [ABSTRACT FROM AUTHOR]

Purpose: The number of accessible central veins (CVs) affects the prognosis of patients with intestinal failure (IF). The loss of residual CVs should be avoided. We, therefore, evaluated the efficacy of a new CV catheter-exchange technique using a subcutaneous fibrous sheath (FS) in pediatric IF patients. Methods: We retrospectively collected the CV catheter (CVC) data of pediatric IF patients managed from January 2009 to December 2019. The data were divided into two groups; Groups 1 (CVCs placed with the FS method) and Group 2 (CVCs placed by the primary or another insertion). The main outcome was the CVC indwelling time. Results: Eighty-five CVCs were analyzed. The FS method was attempted in 47 cases and succeeded in 40 (85%). No significant difference was observed between the groups regarding characteristics. A log-rank test revealed an equivalent CVC indwelling time between the two groups (Group 1: 268 [126–588] days vs. Group 2: 229 [126–387] days, p = 0.256). Conclusions: The FS method is highly recommended for pediatric IF patients, as its attempt showed a high success rate with an indwelling time equivalent to primary insertion. The FS method leads to the prolonged use of a single CV and thereby contributes to improving the outcomes of pediatric IF patients. [ABSTRACT FROM AUTHOR]

Purpose: Congenital diaphragmatic hernia (CDH) is a birth defect of the diaphragm associated with pulmonary hypoplasia. Although genetic factors have been suggested to play a role, the etiology of CDH is still largely unknown. In this study, we analyzed copy number variants (CNVs) using a single-nucleotide polymorphism (SNP) array to examine whether microdeletions contribute to the pathogenesis of this disease.Methods: A total of 28 CDH patients, including 24 isolated and 4 non-isolated cases, were available. We performed CNV analysis using high-resolution SNP arrays (370K, 550K, 660K; Illumina Inc.) and CNstream software. Deletions in loci that have been suggested in previous studies to contain candidate genes affecting CDH were analyzed.Results: We detected 335, 6 and 133 deletions specific for patients in 14 (350K array), 3 (550K) and 11 (660K) cases, respectively. Among these deletions, no segments included the previously suggested candidate genes with the exception of an 18-kb deletion observed in the candidate locus 6q27 in two non-isolated patients. This deleted region contains exon 4 of the t-complex-associated-testis-expressed 3 (TCTE3) gene.Conclusion: Because TCTE3 encodes a putative light chain of the outer dynein arm of cilia and human diseases caused by ciliary dysfunction show various phenotypes including skeletal defect, TCTE3 may be a genetic candidate influencing CDH. [ABSTRACT FROM AUTHOR]

Background/objectives: No definitive treatment strategy has been established for patients with an antenatal diagnosed congenital diaphragmatic hernia (AD-CDH). From 1997 to 2003 in this department fetal stabilization (FS) was administered using both morphine and diazepam via the placenta just before delivery of the fetus by cesarean section. In contrast, from 2004 to the present, a combination of gentle ventilation (GV) and a delayed operation was selected, which was performed when the patient's circulatory stabilization (CS) was achieved.Patients and Methods: This study included 22 patients in the FS group and 16 patients in the GV + CS group, respectively. The outcomes in both groups were compared and the outcome in AD-CDH patients with a patch repaired operation, liver-up or lower lung-to-thorax transverse area ratio (L/T, <0.10) was further investigated in both groups.Results: The overall survival rate (SR) was 93.8% in the GV + CS group and 59.1% in the FS group, respectively (P = 0.04). For the patients with the lower L/T, the SR was 85.7% in GV + CS group and 53.8% in the FS group (P = 0.33). Regarding the patients using a patch and liver-up, the SR in GV + CS group was better than that in the FS group (patch: FS 44.4%, GV +/- CS 87.5%, P = 0.18; liver-up: FS 57.8 and 87.5%, P = 0.30).Conclusion: Our strategy of using GV +/- CS might thus be considered to be more effective than that using FS in the treatment of AD-CDH patients. [ABSTRACT FROM AUTHOR]

Aim: We evaluated a series of late-presenting congenital diaphragmatic hernia (Late-CDH) cases and assessed the reliability and risks of laparoscopic and thoracoscopic approaches for Late-CDH at a single institution. Materials and Methods: From 2005 to 2017, we experienced totally 11 patients with Late-CDH who received endoscopic repairs. We retrospectively surveyed the approach, defect size, operating time, and postoperative outcomes, including recurrence. Results: Eleven patients (Bochdalek, n = 10; Morgagni, n = 1) underwent a total of 14 endoscopic repairs (laparoscopy, n = 10; thoracoscopy, n = 4). The average defect size was ∼3.1 × 1.5 cm. In all 14 endoscopic repairs, patients received intracorporeal interrupted nonabsorbable stitches and extracorporeal knot tying were applied, without the use of an artificial patch. In the laparoscopic repairs, 7 patients received left-handed suturing when closing the diaphragmatic defect, because the reduced viscera lay directly below the posterior rim of the diaphragmatic defect, making it difficult to confirm the rim. In contrast, in the thoracoscopic repairs, the viscera were reduced over the diaphragmatic defect, so the surgeons could easily perform suturing. The average operating time was 172 minutes for laparoscopy and 194 minutes for thoracoscopy. No major intraoperative or postoperative complications occurred in association with either of the approaches. Among the 11 patients, 2 experienced a total of 3 recurrences (all after laparoscopic repairs). Conclusion: Although there were few differences between the laparoscopic and thoracoscopic approaches, because of the technical difficulty of the procedure and the possibility of recurrence with the laparoscopic approach, a thoracoscopic approach may be better for the repair of Late-CDH. [ABSTRACT FROM AUTHOR]

Aspergillus is a widespread fungus in the environment, usually invades through the respiratory tract. Invasive aspergillosis is a fatal disseminated infection in immunocompromised hosts. Appendicitis occurs scarcely in patients with leukemia. We report a case of Aspergillus appendicitis that underwent an urgent appendectomy. An 11-year-old boy received the diagnosis of acute myeloid leukemia, because of the bone pain and results of the bone marrow study. He obtained a complete remission after cancer chemotherapy and received peripheral blood stem cell transplantation from a histocompatible sibling. Leukemia relapsed 5 months post-transplant. Induction therapy with etoposide, cytarabine and mitoxantrone was started on Candida prophylaxis. Fifteen days after the end of chemotherapy, he presented with febrile neutropenia and abdominal pain, that did not respond to broad-spectrum antibiotics. Serum levels of C-reactive protein, β-D-glucan and procalcitonin were unremarkable. Computed tomography scan revealed a swollen appendix and the adjacent tissue inflammation. An urgent appendectomy led to a tentative diagnosis of Aspergillus appendicitis based on the histopathological findings of many fungal hyphal forms. Panfungal polymerase chain reaction using DNA extracted from the lesion determined the pathogen of Aspergillus niger. There was no evidence of invasive aspergillosis. During the prolonged anti-fungal therapy, he achieved a remission of leukemia and underwent the second hematopoietic cell transplantation. To our knowledge, Aspergillus appendicitis was reported to occur in 5 leukemia patients. Four of them survived after appendectomy and one died from intestinal perforation. Early surgical intervention is mandatory for a cure of Aspergillus appendicitis in neutropenic patients on Candida prophylaxis. [ABSTRACT FROM AUTHOR]

Background The diagnosis of biliary atresia ( BA) is still challenging. The aim of this study was to analyze the clinical features and parameters that contribute to a diagnosis of BA. Methods From January 2000 to December 2013, 37 patients who underwent operative cholangiography were evaluated retrospectively. The patients were divided into two groups: a BA group, and a group with other cholestatic diseases (non- BA group). The demographic data and preoperative liver function test results were analyzed. Results Of the 37 patients, 29 patients were confirmed to have BA. Age at operation was not significantly different between the two groups ( P = 0.77). On preoperative liver function tests, only the level of γ-glutamyl transpeptidase (γ- GTP) was significantly higher in the BA group ( P = 0.015). The predominant non- BA disease was inspissated bile syndrome ( IBS). In the IBS patients, the jaundice was relieved after lavage of the biliary tree. Conclusion The preoperative differentiation of cholestasis is difficult based on laboratory data and imaging. Preoperative γ- GTP may be useful for diagnosing BA, but operative cholangiography should be performed when BA is suspected and cannot be ruled out by other methods, given that the most common non- BA disease may be IBS. [ABSTRACT FROM AUTHOR]

Purpose: Very low birth weight infants (VLBWIs) are at risk of surgical intestinal disorders including necrotizing enterocolitis (NEC), focal intestinal perforation (FIP), and meconium-related ileus (MRI). We conducted this study to verify whether the timing of stoma closure and that of enteral nutrition establishment after stoma closure in VLBWIs differ among the most common disorders. Methods: A retrospective multicenter study was conducted at 11 institutes. We reviewed the timing of stoma closure and enteral nutrition establishment in VLBWIs who underwent stoma creation for intestinal disorders. Results: We reviewed the medical records of 73 infants: 21 with NEC, 24 with FIP, and 25 with MRI. The postnatal age at stoma closure was 107 (28-359) days for NEC, 97 (25-302) days for FIP, and 101 (15-264) days for MRI ( p = 0.793), and the postnatal age at establishment of enteral nutrition was 129 (42-381) days for NEC, 117 (41-325) days for FIP, and 128 (25-308) days for MRI ( p = 0.855). The body weights at stoma closure were 1768 (620-3869) g for NEC, 1669 (1100-3040) g for FIP, and 1632 (940-3776) g ( p = 0.614) for MRI. There were no significant differences among the three groups. Conclusions: The present study revealed that the time and body weights at stoma closure and the postoperative restoration of bowel function in VLBWIs did not differ among the three diseases. [ABSTRACT FROM AUTHOR]

Purpose: For 30 years, we have consecutively performed rectal mucosal punch biopsy to diagnose Hirschsprung's disease. The aim of this study was to evaluate the safety of our technique.Methods: Patients with suspected Hirschsprung's disease who underwent punch biopsy, including our original "K-PUNCH" method using an S-moid forceps and non-specific blood-collecting tube at our department and branch hospital between April 1986 and March 2016 were included in the present study. Our punch biopsy technique is characterized by excellent visibility and a direct grasping sensation. The backgrounds and complications of the patients were retrospectively investigated.Results: During this period, 954 patients (median age 4 months; range 1 day-73 years) underwent punch biopsy. Although there were no cases of severe complications (i.e., rectal perforation, infection or full-thickness biopsy), one (0.1%) of the 954 cases in the early period showed liver dysfunction and required transfusion due to bleeding. In addition, inappropriate specimens were obtained in 37 patients (3.9%).Conclusion: Punch biopsy including the "K-PUNCH" method is considered safe and feasible and is associated with a low rate of complications and inappropriate specimen harvesting among patients of all ages. Comorbidities, including the potential for hemorrhage, should always be considered. [ABSTRACT FROM AUTHOR]

Background Determining the cause of bowel obstruction without a history of laparotomy (BO without HL) is difficult and can result in delay of treatment and development of a potentially life-threatening situation. We herein investigated the clinical characteristics of pediatric patients who underwent laparotomy due to BO without HL. Methods All surgical pediatric patients aged <16 age who were diagnosed with BO without HL between January 2004 and September 2014 were included. Etiology of BO, intraoperative findings and postoperative outcome were investigated retrospectively. Results Seventy patients were diagnosed with BO without HL in this period. In these 70 patients, malrotation ( n = 34), intussusception ( n = 19), internal hernia ( n = 6) and Meckel's diverticulum ( n = 5) were predominantly identified. Regarding preoperative definitive diagnosis, prevalence of internal hernia, Meckel's diverticulum or idiopathic volvulus was significantly lower than that of malrotation or intussusception ( P < 0.05). Intraoperatively, the rates of strangulation and bowel resection were 55.7% and 30.0%, respectively. The optimal time for emergency operation in order to avoid strangulated bowel resection was <19 h from onset of symptoms. Conclusions Malrotation and intussusception are major causes of BO without HL in children, but internal hernia, Meckel's diverticulum and idiopathic volvulus should always be taken into account, particularly because of the preoperative diagnostic difficulty and resulting high rate of intestinal resection. In order to avoid resection of the bowel, surgery should be done within 19 h before bowel ischemic change occurs. [ABSTRACT FROM AUTHOR]

Background Transient hyperphosphatasemia (TH), the incidence of which in healthy children is 1.5-2.8%, is associated with a temporary elevation of serum alkaline phosphatase (ALP) without any other liver function test (LFT) abnormalities. Fast α2 band, detected on agarose gel electrophoresis, is known to be a highly sensitive phenomenon in TH. The aim of this study was to elucidate the characteristics of TH after liver transplantation (LT). Methods Five TH patients (6.0%) out of 83 who underwent LT between October 1996 and October 2014, were included in this study. Patient background, duration of TH, peak ALP, time course of ALP and its isoenzyme in TH, other LFT, and imaging results were retrospectively investigated. Results Median patient age at the time of peak ALP during TH was 24 months (range, 16-98 months). Mean duration of TH was 111.0 ± 48.0 days. At 6887 IU/L, median ALP was elevated without any other LFT abnormalities. The first α2 band was detected in all patients, and the band disappeared at the same phase of ALP normalization. All of the patients improved without any treatment. The prevalence of TH was significantly higher in the patients after LT than in age-matched healthy children ( P < 0.05). Conclusions The detection of fast α2 band in the early phase allows for effective diagnosis of TH after LT, and 3-4 month follow up without treatment is feasible. [ABSTRACT FROM AUTHOR]

A massive pulmonary hemorrhage in patients with liver cirrhosis is a life-threatening complication that may result in a contraindication of a liver transplantation because of its high mortality rate. Herein, we present two infant biliary atresia cases that successfully underwent an LDLT that was followed by intensive respiratory care for the pretransplant massive pulmonary hemorrhage. Both cases exhibited severe respiratory failure (minimum PaO2/FiO2; 46 mmHg and 39 mmHg, respectively). To arrest the bleeding, we applied a very high positive pressure ventilation treatment (maximum PIP/ PEEP; 38/14 cmH2O and 55/15 cmH2O, respectively), plasma exchange, several FFP transfusions, and recombinant factor VIIa via intrapulmonary administration. In addition, we used CHDF treatment, applied HFOV transiently, and treated the patient with inhalation of nitric oxide. Although we prepared ECMO for intra-operative use, both cases were successfully managed with conventional mechanical ventilation without using ECMO, which may have worsened the pulmonary hemorrhage due to the use of an anticoagulant. Use of an excessive positive pressure management, although it poses a risk for barotrauma, could be acceptable to arrest the pulmonary bleeding in selected cases of liver failure patients who have no time remaining before LDLT. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study was to evaluate the efficacy of mesenchymal stem cells (MSCs) in a nitrofen-induced congenital diaphragmatic hernia (CDH) rat model. Methods: Pregnant rats were exposed to nitrofen on embryonic day 9.5 (E9.5). MSCs were isolated from the enhanced green fluorescent protein (eGFP) transgenic rat lungs. The MSCs were transplanted into the nitrofen-induced E12.5 rats via the uterine vein, and the E21 lung explants were harvested. The study animals were divided into three: the control group, the nitrofen-induced left CDH (CDH group), and the MSC-treated nitrofen-induced left CDH (MSC-treated CDH group). The specimens were morphologically analyzed using HE and immunohistochemical staining with proliferating cell nuclear antigen (PCNA), surfactant protein-C (SP-C), and α-smooth muscle actin. Results: The alveolar and medial walls of the pulmonary arteries were significantly thinner in the MSC-treated CDH group than in the CDH group. The alveolar air space areas were larger, while PCNA and the SP-C positive cells were significantly higher in the MSC-treated CDH group, than in the CDH group. MSC engraftment was identified on immunohistochemical staining of the GFP in the MSC-treated CDH group. Conclusions: MSC transplantation potentially promotes alveolar and pulmonary artery development, thereby reducing the severity of pulmonary hypoplasia. [ABSTRACT FROM AUTHOR]

Background: Posterolateral or standard axillar incisions for the pediatric thoracic surgery are occasionally associated with poor motor as well as cosmetic results, including chest deformities and large surgical scars. A muscle sparing axillar skin crease incision (MSASCI) was initially proposed by Bianchi et al. (in J Pediatr Surg 33:1798-1800, ) followed by Kalman and Verebely (in Eur J Pediatr Surg 12:226-229, ) resulting in satisfactory cosmetics. However, they performed operations through the third or fourth intercostals space (ICS), therefore the target organs were restricted in the upper two-thirds of the thoracic cavity. Patients and methods: Thoracic surgeries were performed using MSASCI in 27 patients (1-day to 9-year old). There were ten patients with esophageal atresia, seven with congenital cystic adenomatoid malformation, five with pulmonary sequestration, two with mediastinal neuroblastoma, two with right diaphragmatic hernia, and one with pulmonary hypertension. A thoracotomy was performed through the appropriate ICS (from third to eighth). Results: In all patients, the expected procedures, including pulmonary lower lobectomy, were successfully performed by MSASCI throughout the thoracic cavity. A good operational field was easily obtained in neonates and infants. Most of the patients achieved excellent motor and aesthetic outcomes. Conclusions: MSASCI may become the standard approach for the thoracic surgery for small children. [ABSTRACT FROM AUTHOR]

Purpose: Hirschsprung's disease (HD) is usually diagnosed in patients who are under 1 year of age, however, there are still several reports of adult HD cases. We herein analyzed the data of HD patients collected over 30 years according to a nationwide survey in Japan.Methods: The data of HD patients over 15 years of age were thus selected in three phases, namely from 1978 to 1982, from 1988 to 1992, and from 1998 to 2002. A total of 27 patients (0.7%) out of 3,852 were thus analyzed.Results: The male/female was 15/11. The age at diagnosis was as follows: 10 patients were teenagers (37.0%), 14 patients were in their 20s (51.9%), 1 patient was in his 40s (3.7%), and 2 patients were in their 50s (7.4%). The extent of aganglionosis was as follows; the lower rectum: 12 patients (44.4%), the sigmoid colon: 14 patients (51.9%), and the descending colon 1 patient. As a definitive operation, the Duhamel's procedure including Z-shaped anastomosis was performed on 14 patients (51.9%), Swenson's procedure on 5 patients (18.5%), Soave's procedure on 2 patients (7.4%), and Myectomies on 2 patients (7.4%). No mortalities were reported among these cases.Conclusions: Twenty-seven HD patients diagnosed over 15 years of age were analyzed. The number of patient diagnosed over 15 years of age has decreased over time. However, further attention is still required in adult patients who are present with persistent intestinal obstruction of unknown etiology. [ABSTRACT FROM AUTHOR]