Search

To assess mid-/long-term postoperative quality of life (QOL) of esophageal atresia (EA) patients.Modified gastrointestinal quality-of-life index surveys were administered to postoperative EA patients who were at least 7 years old at evaluation to assess three topics about general lifestyle (GL), five topics about EA, and four topics about mental health (MH). For MH, caregivers were also interviewed, but separately. Subjects were divided according to age: children (7-12 years old), teenagers (13-19), and adults (20 and over) and compared according to Foker or Kimura elongation (FK) or bougienage stretching (BS).There were 22 patients evaluated. Responses for GL, EA, and MH did not differ significantly between age groups, but MH responses by caregivers for subjects who were children or teenagers scored significantly lower than responses they made themselves. For primary esophageal elongation technique (PET), age at esophagoesophagostomy was significantly higher in FK. Despite FK scoring 15.1 versus 12.4 for BS during EA evaluation, this difference was not statistically significant.Changes in QOL responses according to age were unremarkable. However, discrepancies in MH indicate that subjects felt better than their caregivers thought. PET did not appear to influence QOL.

Introduction Conscientious follow‐up is essential for bilateral grade 4 hydronephrosis with ureteropelvic junction obstruction to ensure optimal surgical timing. We have reported a case of a male infant who required emergent urinary drainage due to severe bilateral ureteropelvic junction obstruction‐derived acute renal failure. Case presentation Bilateral grade 4 hydronephrosis was diagnosed in a male neonate. Vesicoureteral reflux was ruled out. Two years after the initial diagnosis, he developed acute renal failure and underwent bilateral emergent urinary drainage, followed by multiple urinary tract reconstructions against left ureterovesical junction stenosis and bilateral ureteropelvic junction obstruction. The postoperative renogram demonstrated a bilateral nonobstructive pattern. Conclusion Bilateral emergency drainage for acute renal failure was successful without hemodialysis. Unilateral drainage or pyeloplasty should be planned early for bilateral grade 4 hydronephrosis with ureteropelvic junction obstruction to avoid lethal events if the obstruction pattern with decreased split renal function is

To assess mid-/long-term quality of life (QOL) of total colonic aganglionosis (TCA) patients.Modified pre-existing QOL assessment tools for general lifestyle (GL), bowel function (BF), and mental health (MH) were administered to postoperative TCA patients from five institutions, who were at least 7 years old to compare Duhamel (with pouch) and Swenson/Soave (without pouch) techniques between children (Ch 7-12 years old), teenagers (Tn 13-19), and adults (Ad 20 and over). For MH, caregivers were also interviewed, but separately. Maximum scores were 12 for GL/MH and 18 for BF.There were 32 subjects. GL and BF scores increased significantly from Ch (GL 4.8 ± 2.5, BF: 11.3 ± 4.6) to Tn (GL 7.8 ± 2.6, BF 16.2 ± 3.0); scores for MH did not change significantly. Mean caregiver MH scores were significantly lower than mean subject MH scores for all age groups (subject scores: 10.1, 10.7, 10.7 versus caregiver scores: 6.8, 7.8, 8.1 for Ch, Tn, Ad, respectively). PT technique/presence of a pouch did not influence the incidence of enterocolitis or QOL scores.MH responses showed subjects felt better than caregivers believed. This discrepancy could cause conflict despite steadily improving GL/BF. QOL was unaffected by PT technique/presence of a pouch.

The incidence of perforation during antibiotic therapy (AT) of children triaged as non-complicated acute appendicitis (NC-Ap) was investigated. Abdominal ultrasonography (US) and/or computed tomography (CT) scans from cases of perforation identified at appendectomy for failed AT were reassessed blindly by a panel of board-certified specialists for any evidence of pre-AT morbidity suggestive of perforation. Of 521 cases triaged as NC-Ap, symptoms resolved with AT in 452 cases (86.8%). All 69/521 (13.2%) cases with persistent symptoms had urgent appendectomy, and 12/521 (2.3%) were found to have perforated. Blind reassessment of US and/or CT scans from these cases identified seven with evidence of perforation when they were triaged as NC-Ap. Thus, the actual incidence of perforation during AT for NC-Ap was actually 12–7 = 5/521 (0.95%). Perforation is generally believed to be a complication of AT, but inappropriate triaging of cases for AT can bias results by artificially inflating the number of perforations, in this study, by more than double. We are the first to assess the unbiased incidence of perforation during AT for NC-Ap, by reassessing pre-AT US and/or CT scans. The incidence of perforation during AT is actually negligible.

We developed treatment guidelines (TGs) for appropriate transitional care of the genitourinary system in patients with persistent cloaca (PC), cloacal exstrophy (CE), or Mayer-Rokitansky-Küster-Häuser syndrome (MRKH). These TGs are in accordance with the Medical Information Network Distribution Service (Minds), published in 2014 in Japan. Clinical questions (CQs) concerning treatment outcomes of the genitourinary system, pregnancy and delivery, and quality of life in adulthood were prepared as six themes for PC and CE and five themes for MRKH. We were able to publish statements on chronic renal dysfunction, hydrometrocolpos, and pregnancy, based on four CQs about PC, four about CE, and two about MRKH, respectively. However, due to the paucity of proper manuscripts, we were unable to make conclusions about the correct timing and method of vaginoplasty for patients with PC, CE, and MRKH or the usefulness of early bladder closure for patients with CE. These TGs may help clarify the current treatments for PC, CE, and MRKH in childhood, which have been carried out on an institutional basis. To improve clinical outcomes, systematic clinical trials revealing comprehensive clinical data of the urinary and reproductive systems, especially the length of the common channel in PC, are essential.

To document the recovery of bowel function (BF) in children after transperitoneal (TP) or retroperitoneal (RP) laparoscopic pyeloplasty. Data were obtained retrospectively from four centers between 2008 and 2019 for TP (n = 51) and RP (n = 58). Each surgeon chose which technique to perform. Subject demographics were not significantly different. Differences in operative times were not significant (RP: 241 min versus TP: 225 min). Mean duration/requirement for postoperative epidural/intravenous analgesia were not significantly different (TP: 1.4 days versus RP: 1.3 days) and (TP: 66.7% versus RP: 67.2%), respectively. Postoperative nasogastric (NG) intubation was more common in RP (TP: 19.6% versus RP: 44.8%; p

Background The management of large abdominal wall defects, such as omphalocele or gastroschisis, remains a challenge for pediatric surgeons. Though several techniques have been described to repair those conditions, there is no procedure considered to be the standard worldwide. We report an infant girl with a giant ventral hernia after staged surgery for omphalocele in whom delayed closure of a large abdominal wall defect was performed successfully using “endoscopic component separation technique (ECST)” without serious complications. Case presentation A baby girl was admitted to our hospital because of a giant omphalocele, which had been prenatally diagnosed. The omphalocele was supraumbilical and included the entire liver. After staged surgery, a large abdominal wall defect was closed by skin, creating a giant ventral hernia. We performed endoscopic separation component technique (ECST) for the closure of her abdominal wall defect when she was 11 months of age. ECST was initiated with placement of a 5.0-mm port just above the inguinal ligament and under the external oblique muscle. The space between the external and internal oblique muscles was created by the insufflation pressure, and a second 5.0-mm port was placed at 1.0 cm below the inferior edge of the rib into the space. As the further dissection was carried, the aponeurosis of the external oblique muscle was identified as a white line, running vertically from the epigastrium to inguinal ligament. It was transected longitudinally using electrocautery over its full length. The same procedure was performed on the contralateral side and the abdominal wall was successfully closed. Postoperative course was uneventful. Conclusions The technique of ECST, described here, is simple and safe for infants, and the cosmetic result is satisfactory.

BACKGROUND Clonal evolution of malignancy is a complex process related to intratumoral heterogeneity, as recent studies have also demonstrated in rhabdomyosarcoma. The purpose of this study is to present a distinct clonal feature of a case with anaplastic embryonal type rhabdomyosarcoma (ERMS) using molecular analysis. METHODS A five-year-old girl developed a metastatic pelvic tumor. We cultured neoplastic cells isolated from the biopsy sample. Next, to characterize the current case, we analyzed the biopsy sample, autopsy sample, and established cell line using combined modalities, including histopathological, cytogenetic, and molecular assay. We also undertook the backtrack mutation-specific polymerase chain reaction to reveal clonal composition. RESULTS The histology of the biopsy sample was consistent with ERMS with focal anaplasia. We established a permanently growing cell line, ICH-ERMS-1, from the biopsy sample. On molecular analysis, the biopsied tissue revealed a missense mutation at codon 245 of TP53. In contrast, the autopsy tumor tissue and the cell line established from the biopsied tissue showed a missense mutation at codon 248. A backtrack study using mutation-specific polymerase chain reaction detected a TP53 codon 248 mutation in the original biopsy sample. All the specimens examined had a missense mutation at PTPN11 codon 69. CONCLUSIONS This study highlights intratumoral heterogeneity and distinct clonal change related to the functional context in our anaplastic ERMS case, supporting the concept of intratumoral heterogeneity and clonal evolution. It requires further case collection to reveal whether p14ARF-p53-MDM2 tumor suppressor pathway alteration, considered a late event in ERMS tumorigenesis, is responsible for anaplasia in ERMS.

A 30-day-old female infant presented with a cystic lesion at the porta hepatis. The cyst was first detected on a prenatal ultrasound at 16 weeks of gestation and diagnosed as congenital biliary dilatation. The size of the cyst did not change over the course of gestation and the infant's liver function was stable after birth, although she had a persisting yellowish stool. Surgical cholangiography was performed at 30 days, and a complete obstruction of the hepatic hilum was identified as a type IIId cyst. However, cholangitis recurred at 10 months post-surgery, with marked dilation of the right and left hepatic ducts. Right cyst-intestinal anastomosis and left cyst-gastric anastomosis were performed. Cyst recurrence is sometimes a complication in patients with type III biliary atresia who undergo the Kasai procedure. The mechanisms of cyst formation are still unknown, and there is no consensus regarding the treatment. Our case suggests that cysto-enterstomy is a feasible treatment for recurrent cysts.

Highlights • Food allergy in neonates with secondary necrotizing enterocolitis is extremely rare. • No typical risk factor for necrotizing enterocolitis due to food allergy was found. • Methicillin-resistant Staphylococcus aureus in the stool may be a risk factor., Introduction The incidence of gastrointestinal food allergy (FA) in neonates is increasing. Despite this, cases of patients with gastrointestinal FA who develop necrotizing enterocolitis (NEC) requiring laparotomy are extremely rare. Presentation of case We describe two cases that presented with bloody stool with a probable diagnosis of FA as eosinophils were positive in the stool at onset. Both cases failed conservative treatment. Jejunostomy and ileostomy were performed in both cases due to secondary NEC with underlying acute FA. Post-surgery, raised peripheral blood eosinophil count, presence of cow’s milk-specific IgE antibody and positive allergen-specific lymphocyte stimulation test were found. Stoma closure were performed 3 and 1 months later in both cases. Postoperative recovery was uneventful. Discussion A few reports have not identified risk factors for NEC secondary to FA. Thrombocytopenia and rise in C-reactive protein (CRP) levels 2 days after the development of FA may be suggestive of FA with NEC. Methicillin-resistant Staphylococcus aureus (MRSA) was detected in the fecal culture of both patients at the time of the onset of NEC. The toxic antigen produced by MRSA may cause activation of milk-protein-primed T cells and exacerbate FA. Conclusion The decrease of platelet levels and rise in CRP may indicate the development of secondary NEC in patients with FA. Additionally, MRSA detected in the fecal culture also may be a risk factor for NEC through the activation of cellular immunity reaction pathways.

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. 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. 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. 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.

We present a first report of the long-term follow-up of biliary atresia (BA) patients who became anicteric with the native liver (ANL; total bilirubin1.5mg/dL) after redo-Kasai.Forty-six redo-Kasai cases (1984-2015) were the subjects for this study. ANL ratios were determined using the Kaplan-Meier estimate.BA type was I (n=3), II (n=1), and III (n=42). Mean ages (initial/redo) at Kasai were 60.3/231.9days, respectively. Jaundice persisted after the initial Kasai in 24/46 cases while 22 had recurrence of jaundice after initially becoming anicteric. After redo, 5/24 of the persistent jaundice cases and 14/22 of the initially anicteric cases became anicteric (p.05). Of these 19, 7 (one type I, six type III) are currently ANL while the remaining 12 had LTx or died. Morbidity/Complications documented in the 7 post-redo ANL cases after a mean follow-up of 16.7years (range: 10.0-31.1) included 4 episodes of cholangitis, 3 episodes of portal hypertension, 4 episodes of esophageal varices, 3 episodes of splenomegaly, one splenectomy, and 4 episodes of thrombocytopenia.Ours is the first long-term follow-up study of redo-Kasai cases. We found that the ANL ratio after redo-Kasai was low at 7/46 (15.2%) and that ANL were prone to multiple morbidity.Retrospective comparative study, level III.

Aim The purpose of this study was to determine the incidence of ureteric obstruction (UB) and requirement for Cohen antireflux surgery (CAS) after Deflux Ⓡ treatment (DT) for vesicoureteric reflux (VUR). Methods Between 2011 and 2017, 494 ureters (VUR severity ≤ grade III: N=291 or >grade IV: N=203) were treated by DT at a mean age of 4.5 (range: 0.2–24) years. Epidural Catheter Assistance (ECA) was used to exclude UB by injecting diluted indigo carmine solution (1–3mL) into an epidural catheter inserted into a ureter after DT and confirming dye flow within 15min. ECA+: N=181 ureters; ECA−: N=313 ureters. Results In ECA+, UB was detected in 5/181 (2.7%) ureters (grade II: N=1, III: N=3, and IV: N=1) treated by leaving the ECA catheter in situ overnight (N=4) or double J stent (DJS) insertion for 1month (N=2). After mean follow-up of 1.9years, one grade III DJS case has residual grade II VUR. In ECA−, 3/313 (0.9%) cases developed UB. One resolved, and one required DJS. CAS was required for 17/494 (3.4%) ureters and hindered by DT in 5/17 (29.4%) ureters. All are sequelae-free after mean follow-up of 1.8years. Conclusions UB may be more frequent than reported (3.3% versus 0.6%–1.8%). ECA identifies potential UB. Type of study Treatment study. Level of evidence Level III.

A 6-year-old girl underwent ureterocystoneostomy (UCN) because of left flank pain due to delayed onset of ureteral stenosis one and a half years after endoscopic dextranomer/hyaluronic acid copolymer (Deflux) injection for the treatment of vesicoureteral reflux (VUR). Histopathological examination indicated chronic inflammation with abundant eosinophils characteristic of a reaction to Deflux. Several autoimmune diseases developed during the treatment for ureteral stenosis. First, 2 weeks prior to the onset of left flank pain, she was diagnosed as having systemic lupus erythematosus. Finally, she died of pulmonary hemorrhage due to thrombotic thrombocytopenic purpura 4 months after UCN. The fatal outcome in this case was suspected to be caused by autoimmune syndrome induced by adjuvants, which in this case was the hyaluronic acid polymer injected into the ureteric orifice for the treatment of VUR.

We report two patients with chronic granulomatous disease (CGD). The first patient presented with granulomatous colitis and pulmonary aspergillosis, and the second patient with liver abscess and restrictive pulmonary disorder. Both patients underwent allogeneic hematopoietic stem cell transplantation, the first from an HLA-matched sibling donor, and the second from an HLA-matched unrelated donor, after preconditioning with fludarabine, anti-thymocyte globulin, cyclophosphamide, and total-body irradiation of 3 Gy. The engraftment was prompt and the regimen-related toxicity was mild. The patients are able to return to their daily lives with full donor chimerism, although the second patient underwent a living-related-donor orthotopic liver transplantation from his mother for chronic liver graft-versus-host disease. The conditioning regimen we used was feasible and applicable to patients with CGD accompanied by inflammatory disease and severe infection.

The identification of novel synthetic ligands for G protein-coupled receptors (GPCRs) is important not only for understanding human physiology, but also for the development of novel drugs, especially for orphan GPCRs for which endogenous ligands are unknown. One of the orphan GPCR subfamilies, Super conserved Receptor Expressed in Brain (SREB), consists of GPR27, GPR85 and GPR173 and is expressed in the central nervous system. We report herein the identification of inverse agonists for the SREB family without their agonists. We carried out an in vitro screening of 5472 chemical compounds from the RIKEN NPDepo chemical library. The binding of [(35) S]GTPγS to the GPR173-Gsα fusion protein expressed in Sf9 cells was measured and resulted in the identification of 8 novel GPR173 inverse agonists. The most potent compound showed an IC50 of approximately 8 μm. The identified compounds were also antagonists for other SREB members, GPR27 and GPR85. These results indicated that the SREB family could couple Gs-type G proteins, and SREB-Gsα fusion proteins showed significant constitutive activities. Moreover, a molecular model of GPR173 was constructed using the screening results. The combination of computational and biological methods will provide a unique approach to ligand identification for orphan GPCRs and brain research.

The management of appendiceal masses (AM) in children remains controversial. In this study, we evaluated primary laparoscopic drainage (PLD) for efficacy.Eleven (11) consecutive cases of AM (mean age, 8.1 +/- 2.8 years) treated between 2000 and 2004 were the subjects for this study. All had PLD on presentation. If the appendix was seen easily seen after PLD, a laparoscopic appendectomy (LA) was also performed.Eight (8) patients underwent PLD alone (LD group) and 3 underwent PLD and LA (LDLA group). In the LD group, the mean operating time was 87.9 +/- 23.2 minutes, oral feeding was commenced after a mean of 2.3 +/- 0.8 days, patients became afebrile within 4.3 +/- 3.1 days, intravenous antibiotics were ceased after 5.3 +/- 3.1 days, C-reactive protein normalized within 13.6 +/- 4.2 days, drains were removed within 4.0 +/- 1.3 days, and hospital stay ranged from 7 to 15 days. There were no intra- or postoperative complications related to the PLD procedure. Interval LA was performed 6.8 +/- 5.8 months after PLD in 6 of 8 LD group patients and was not performed in the remaining 2 owing to parental refusal. In the LDLA group, operating time ranged from 125 to 150 minutes, and oral feeding commenced 4, 5, and 5 days after LA, respectively. One (1) patient developed an adhesive bowel obstruction after LA, which resolved with conservative therapy; in the remaining 2, there were no complications. All 11 patients are well after a mean follow-up period of 3.7 +/- 1.0 years. A histologic examination of the excised appendices showed mild to severe inflammation.We recommend that PLD be adopted for the primary management of appendiceal masses, as it would appear to be simple, safe, and effective.

The aim of this study was to describe our improved technique for stoma closure, laparoscopy-assisted stoma closure (LASC).Eleven (11) children had LASC at our institute during 2005. Their ages at LASC ranged from 4 to 23 months and their body weight ranged from 3.4 to 10.0 kg. Under general anesthesia, a 5-mm trocar was inserted through an infraumbilical, left-lower, or upper quadrant incision, and laparoscopy was used to observe the bowels, the stoma, the line of separation, and any adhesions. Externally, an incision was made around the stoma circumferentially, and a pair of mosquito forceps was inserted into the abdomen along the attachment between the stoma and the abdominal wall where no intra-abdominal adhesions were present, and the tips of the mosquito forceps were used to free the stoma along the proposed line of separation. Electrocautery was used for hemostasis and for completing the separation. After the stoma was taken down, the bowel was anastomosed and the abdomen closed in layers.All stomas were taken down easily without any complications in all cases. The average time for each stomal separation, from incising around the stoma until the stoma was taken down, was 23.1 minutes (range, 17-42). Injury to the abdominal wall musculature was minimal. There was 1 case of postoperative small bowel obstruction and no wound infection or incisional herniation.Although our experience is limited to only 11 patients, our LASC procedure appears to be an effective option for stoma closure.

The aim of this study is to investigate the optimal treatment for lymphangioma in children by analyzing the effectiveness and complications of treatment modalities.We reviewed 128 patients with lymphangioma treated at our institution between 1979 and 2005. Periods of treatment were divided arbitrarily into 2 groups: period I, from 1979 to 1988 (n = 53); and period II, from 1989 to 2005 (n = 75). According to radiological appearance, patients were grouped into 4 types: single cystic (SI; n = 23), macrocystic (MA; n = 11), microcystic (MI; n = 69), and cavernous (CA; n = 25).Sclerotherapy as primary treatment was performed in only 2 patients (3.8%) in period I using bleomycin but increased significantly in period II to 48 patients (64.0%) using OK-432 (P.01). Nevertheless, primary surgical excision (69/78 patients, 88.5%) was significantly more successful than sclerotherapy (32/50, 64.0%) (P.01). The following are the locations and types of lesions: head/neck (n = 69; SI, 11; MA, 5; MI, 42; CA, 11), trunk (n = 34; SI, 6; MA, 6; MI, 15; CA, 7), and extremities/other (n = 25; SI, 6; MI, 12; CA, 7). The effectiveness of sclerotherapy in SI, MA, MI, and CA types was 90.9%, 100%, 68.0%, and 10.0%, respectively. Seventeen patients (SI, 1; MI, 8; CA, 8) who received primary sclerotherapy required surgical excision with good outcome. Complications after primary surgical excision were more serious compared with sclerotherapy.Sclerotherapy with OK-432 was not as effective as reported in the literature. We recommend OK-432 injection therapy alone for SI and MA types and surgical excision after pretreatment with OK-432 for MI and CA types.

We modified conventional hepatic portoenterostomy by pulling the remnant fibrous mass caudally and placing 5 or 6 numbered and individually clamped absorbable 5-0 double-needle sutures horizontally in the liver surface of the posterior side of the remnant fibrous mass before excision and apposing the posterior and anterior margins of the hepatic portoenterostomy to resemble a flattened isosceles triangle. Our technique was used to treat 14 cases of biliary atresia. Good postoperative biliary excretion (serum total bilirubin

Prednisolone is used routinely after portoenterostomy (PE) in patients with biliary atresia (BA). The authors reviewed their patients with BA post-PE to assess prednisolone protocols.Severity of fibrosis at PE (moderate or severe), age at PE (30-70 days), size of bile ductules in the fibrotic biliary remnant at the porta hepatis (100 microm), and type of BA (uncorrectable type) were used as criteria for selecting 63 subjects from our patients with BA post-PE. Subjects were divided into 5 groups according to prednisolone dosage: group 1, no prednisolone; groups 2 to 4, single courses of intravenous prednisolone commencing on day 7 post-PE administered in decreasing dose for 3 days each as follows: group 2, 6, 4, and 2 mg; group 3, 10, 5, and 2.5 mg; group 4, 20, 15, 10, 5, and 2.5 mg; group 5, same as group 4, but stool color was used to monitor bile excretion and a course was restarted from 20 mg whenever stools began to turn pale. If necessary, single courses were repeated until serum total bilirubin was less than 2.0 mg/dL. Protocol efficacy was assessed by comparing the number of patients who became jaundice free, the period taken to become jaundice free, and the incidence of side effects related to prednisolone.The number of patients who became jaundice free in the no prednisolone group (group 1, 7/12 or 58.3%) was not significantly different from the number in the single-course groups (group 2, 8/12 or 66.6%; group 3, 10/13 or 76.9%; and group 4, 11/15 or 73.3%). The number in the stool-monitored group (group 5, 10/11 or 90.9%) was significantly greater (P.05). The mean period taken to become jaundice free in group 1 (82.6 +/- 29.1 days) was not significantly different from the single-course groups (group 2, 74.5 +/- 29.3 days; group 3, 49.6 +/- 19.8 days; and group 4, 48.3 +/- 26.0 days). The mean period taken in the stool-monitored group (group 5, 33.3 +/- 6.4 days) was significantly shorter (P.05). The number of subjects who developed cholangitis after becoming jaundice free was not significantly different (group 1, 2/7; group 2, 2/8; group 3, 2/10; group 4, 2/11; group 5, 2/10). There were no prednisolone-related complications identified in any subject.These results provide strong evidence that large-dose prednisolone therapy with stool color monitoring of bile flow has a positive impact on the time taken for patients with BA post-PE to become jaundice free and the number of patients who remain jaundice free.

We report two rare cases of botryoid Wilms' tumor (BWT) occupying the renal collecting system with no macroscopic parenchymal mass. In case 1, a 3-year-old boy presented with a mass in the right flank, low-grade fever, abdominal pain, and macrohematuria. Radiology revealed an enlarged right kidney with a heterogeneous mass occupying a large part of the dilated renal calyx, pelvis, and ureter. Radical right nephroureterectomy was performed. The histopathologic diagnosis was nephroblastoma, and the pedicle of the tumor was attached to the renal parenchyma near the pelvic wall. In case 2, a 2-year-old boy presented with macrohematuria, and the clinical course was almost the same as in case 1. No local recurrence or metastatic spread has been detected for 4 years postoperatively in case 1 and for 9 months postoperatively in case 2.

Homozygous mutant Ncx/Hox11L.1-deficient (Ncx-/-) mice develop mega-ileo-ceco-colon (mega-ICC) with a caliber change in the proximal colon. The authors investigated the mechanism of intestinal dysmotility in these mice.Five-week-old Ncx-/- mice with mega ICC were compared with age-matched BDF1 control mice. Jejunum, ileum, and colon were excised from all mice and 1.0-cm-long strips of each organ, each with a resting tension of 0.5g, were suspended in an organ bath filled with Tyrode's solution at 37 degrees C and bubbled with a mixture of 95% oxygen and 5% carbon dioxide. Contractile responses to acetylcholine chloride (ACh), histamine, serotonin, and barium chloride (BaCl2) were recorded isometrically.For ACh, Ncx-/- mice had decreased distal colon circular muscle contraction only at lower doses and decreased distal colon longitudinal muscle contraction for all doses compared with controls (P.05 or P.01). In the proximal colon, Ncx-/- mice had increased circular muscle contraction only at higher doses and decreased longitudinal muscle contraction only at lower doses compared with controls (P.01 or P.05). ACh did not affect jejunum, and there were no significant effects on ileum. There was no response to histamine and serotonin by any part of the bowel, and the response to BaCl2 was the same for both Ncx-/- mice and controls.Only ACh differentially affected muscle contraction in Ncx-/- mice in the proximal and distal colon. Thus, ACh is implicated in causing the bowel dysmotility seen in Ncx-/- mice and human IND.

Purpose: Forme fruste choledochal cyst (FFCC) is a choledochal cyst that has minimal or no dilatation of the extrahepatic bile duct (EHBD) and is associated with pancreaticobiliary malunion (PBMU). The authors reviewed the long-term outcome of their patients with FFCC. Methods: Inpatient and outpatient records of 281 patients with choledochal cyst were reviewed. In this study, minimal dilatation of the EHBD was defined as its maximum diameter being less than 10 mm. Results: There were 17 cases of FFCC identified. In all 17 patients, PBMU was present. The mean age at EHBD excision was 2.9 years. Fourteen patients had hepatico-jejunostomy, and three had hepatico-duodenostomy. The histology of the excised EHBD showed mucosal ulceration/sloughing (in 35.3% patients), fibrosis (52.9%), and inflammatory cell infiltration (41.2%). Over a mean postoperative follow-up period of 9.8 years, there have been no episodes of cholangitis or anastomotic stricture formation. Conclusions: The treatment of choice for FFCC in children is EHBD excision and hepatico-jejunostomy. There is little surgical morbidity if performed carefully.

This study aimed to clarify the role of complement activation in fibrogenesis in BA.In total, 27 paraffin-embedded liver biopsy samples were immunohistochemically analyzed using C4d polyclonal antibody, vascular cell adhesion molecule-1 (VCAM-1), and CD45. The liver samples were obtained from 25 patients during Kasai operation, and two additional specimens were obtained from 2 patients by needle biopsy later at the time of liver function deterioration. The degree of liver fibrosis was histologically graded 1-3.Among the 25 samples, 9 showed C4d-positive immunostaining localized on the endothelia of a few portal veins in the portal tract. The degree of fibrosis was correlated with C4d staining (p = 0.025). The age at Kasai operation correlated with the degree of fibrosis and the C4d positivity. Two needle biopsy samples were positive for C4d. Among 13 samples submitted for VCAM-1 staining, 2 negative samples were C4d negative and all positive C4d samples were VCAM-1 positive with CD45 mononuclear cell infiltration.These findings suggest that ongoing cirrhosis could be a result of progressive "vasculopathy" of the portal vein caused by humoral and cell-mediated immune interaction.

To study the surgical treatment and outcome of hydronephrosis secondary to pelviureteric junction obstruction (PUJS) that is so massive that the renal pelvis crosses over the vertebral column [mega-hydronephrosis (MH)]. Of the 40 cases of PUJS we treated over the past 5 years, 6 cases had MH secondary to PUJS (MH-PUJS) in 6 renal units and were reviewed. Incidence of MH was 15.0%. All had Anderson-Hynes dismembered pyeloplasty to treat obstructive renal pattern on technetium-99m diethylenetriaminepentaacetic acid (DTPA) scans in five and gait disturbance due to MH in one. The contralateral kidney was normal in five and dysplastic in one. Three had nephrostomy before pyeloplasty. Preoperative DTPA scans showed good renal function in four and poor in two. Plication of the entire dilated, lax renal parenchyma ("nephroplication") was performed in three to decrease the intra-renal pelvic cavity. Prenatal diagnosis was performed in three cases. Mean age at pyeloplasty was 3.5 years and mean duration of follow-up was 1.5 years. Postoperatively, two of the three who did not have nephroplication had prolonged but not obstructed renal pattern on DTPA scans, and the other case who did not have nephroplication had nephrectomy for non-functioning kidney caused by recurrent pyelonephritis 2 years after pyeloplasty. In the three who had nephroplication, DTPA scans showed acceptable to relatively good passage in all cases. The surgical treatment of MH-PUJS is challenging with no fixed guidelines. However, nephroplication may be useful during pyeloplasty to decrease the size of MH-PUJS.

An 18-year-old girl who had undergone excision of a choledochal cyst and Roux-en-Y hepatico-jejunostomy at another hospital when 23 months old was referred to our department because of recurrent cholangitis. Radiological investigations showed stones lying in minimally dilated, right posterior intrahepatic bile ducts (IHBD). At laparotomy, the hepatico-jejunostomy site was incised, and a flexible endoscope inserted into the IHBD. Multiple stones packed in the IHBD were easily fragmented using an electro-hydraulic lithotripsy (EHL) device inserted through the endoscope, and removed. There were no EHL-related complications, and her postoperative progress was uneventful. She is currently well with no episodes of cholangitis after a follow-up period of 3 years. EHL is a simple, effective alternative method for removing IHBD stones after choledochal cyst excision. To the best of our knowledge, this is the first report of EHL being used to remove stones that developed in the IHBD after choledochal cyst excision.

There has been no report describing suspected prenatal pyriform sinus (PS) cyst and fistula. We report a case suspected by prenatal ultrasonography and fetal MRI. A large cystic mass was found in the left neck of the fetus. After the baby was born, preoperative laryngoscopic catheterization of the fistula tract was used to confirm the diagnosis and greatly facilitated the identification and excision of the PS cyst and fistula.

We investigate the incidence, treatment, and implications of intrahepatic bile duct (IHBD) dilatation debris in 42 patients with choledochal cyst treated over a recent 8-year period. Mean age at cyst excision was 6.9 years, and mean follow-up period was 5.4 years. Intraoperative endoscopy (IOE) was performed at the time of cyst excision using a pediatric cystoscope, which identified IHBD debris in 11/42 (26.2%), and massive debris in 4/42 (9.5%). In all cases, IHBD debris was successfully washed out with normal saline through the cystoscope. There was no postoperative IHBD stone formation or cholangitis except for one case of cholangitis in a patient undergoing chemotherapy for acute myelocytic leukemia. Debris left in the IHBD during cyst excision is probably one of the primary causes of postoperative IHBD stone formation and can be prevented by washing during IOE.

Purpose: The aim of this study was to review the outcome of a protocol the authors proposed for preventing Roux-en-Y hepatico-jejunostomy (RYHJ) related complications. Methods: In 1986 the authors adopted the following protocol for improving RYHJ to minimize risks for RY-related complications: (1) end-to-end anastomosis if possible; (2) if end-to-side anastomosis is unavoidable, the common hepatic duct should be anastomosed as close as possible to the closed end of the blind pouch (BP); (3) careful selection of the vascular supply to the RY jejunal limb; (4) length of the RY jejunal limb should not be predetermined, but individualized; (5) approximate the native jejunum to the RY limb. The authors reviewed 92 children who had surgery for CC (mean age at surgery, 4.1 years) at our institution between 1986 and 2002 to assess the effectiveness of their recommendations. Results: After the adoption of the authors' recommendations for RYHJ, they have experienced no RYHJ-related complications after a mean follow-up period of 7.6 years (range, 4 months to 16 years). Conclusions: RYHJ in children is different from that in adults, because the RY limb or BP can grow and elongate as the child grows. The authors' recommendations appear to have successfully prevented RY-related complications up to the time of this review, and they highly recommend their use.

Cloacal dysgenesis sequence (CDS) is a rare and lethal malformation. We report such a case of long-term survival, currently to 12 years of age. In the fetal period, she received a timely placement of vesico-amniotic shunt for a megabladder due to a severe urethral obstruction. Postnatally, cystostomy and colostomy were created because of no perineal opening of urethra, vagina, and anus. Anorectoplasty, construction of efferent conduit, and colostomy closure were performed at 4 years of age. Ileovaginoplasty and ileovesicostomy which was a Mitrofanoff-type of conduit, and labioplasty were performed at the age of 11 years. To the best of our knowledge, only five survivors with CDS over 1 year of age have been reported.

Urethral duplication is a rare congenital anomaly with various clinical presentations, and multiple techniques have been described for its repair. We report a 1-year-old boy with hypospadiac urethral duplication who presented with double urinary stream. Voiding cystourethrography, retrograde urethrography, and cystourethroscopy showed the normal-caliber ventral urethra was dominant and the distal dorsal (non-dominant) urethra had a good caliber. Urethral reconstruction was performed with an incision of the adjoining walls of the both urethra in a side-to-side urethrourethrostomy fashion.

Laparoscopy-assisted stoma closure (LASC; n = 28) and conventional open closure (CSC; n = 24) were compared by assessing outcome in 52 consecutive cases of stoma closure (SC) in children.

Gastrointestinal stromal tumors (GIST) are c-KIT positive neoplasm that occur predominantly in adults. A 13-year-old boy who presented with massive gastrointestinal hemorrhage was subsequently diagnosed with GIST of the stomach. The tumor originated from the lesser curvature with a narrow base and no infiltration into surrounding organs. Two metastatic lesions in the liver were also found. Total excision of the gastric lesion was performed followed by adjuvant imatinib mesylate chemotherapy. He has had no signs of recurrence during 8 months of follow-up. GIST in children are rare and their behavior is notoriously difficult to predict. We report our experience and review the literature.