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Background: Creating obstructive uropathy (OU) during glomerulogenesis in the fetal lamb results in multicystic dysplastic kidney (MCDK) at term. We explored this using immunohistochemical techniques., Method: OU was created in fetal lambs at 60-day gestation, ligating the urethra and urachus. The kidneys of MCDK lambs, 60-day gestation fetal lambs, full-term lamb (145 days), term sham-operated lambs, and adult ewes were evaluated by HE staining, and immunohistochemistry with paired box genes 2 (PAX2) and CD10., Results: Multiple cysts were found in the MCDK model. CD10 was expressed in proximal tubular epithelial cells, glomerular epithelial cells, and medullary stromal cells in the kidneys of 60-day gestation fetal lambs and full-term lambs and adult ewes. PAX2 expression was found in ureteric buds, C- and S-shaped bodies, epithelial cells of collecting ducts, and Bowman's capsule of fetal kidneys at 60-day gestation, but only in the collecting ducts of full-term fetal lambs and adult ewes. Both CD10 and PAX2 were expressed in the cystic epithelial cells of the MCDK model., Discussion: PAX2 expression in cystic epithelial cells suggests that cyst formation is associated with disturbed down-regulation of PAX2 in the nephrogenic zone epithelial cells during the renal development in the OU model., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: In biliary atresia (BA), the ultrasonic triangular cord (TC) sign is positive at ≥ 3 mm, but sometimes there is BA even if it is ≤ 3 mm. For improving the ultrasonographic diagnosis, we have established a new evaluation, adding the ratio of the anterior/posterior thickness (TC ratio) in the hyperechoic area and the presence of a cystic lesion in the triangular cord (TCC)., Methods: We examined 24 cases of suspected BA who demonstrated acholic stools from 2006 to 2020. We retrospectively reviewed the timing of ultrasonographic diagnosis, the gallbladder diameter, gallbladder mucosal irregularity, the TC sign, TCC, and the TC ratio., Results: In the BA group (n = 10) vs the Non-BA group (n = 14), the age at ultrasonography was 75 ± 41.7 vs. 81 ± 39.1 days (p = 0.72), the gallbladder diameter was 12.1 ± 9.7 vs. 24.2 ± 6.96 mm (p = 0.02), irregularity of gallbladder mucosa was 7 cases vs. 1 case (p < 0.01), and TC sign was 3.9 ± 1.3 vs. 2.0 ± 0.49 mm (p = 0.01), respectively. TCC was observed in 8/10 cases in the BA group and none in the Non-BA group (p < 0.01). TC ratio was 3.40 ± 0.68 (BA group) and 1.59 ± 0.41 (Non-BA group) (p < 0.01)., Conclusion: The ultrasonic TC ratio improves the diagnostic accuracy of BA. TCC is a specific finding in the BA group., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: We created abdominal wall defects (AWD) in fetal lambs to investigate possible causes of scoliosis., Methods: We incised the upper abdominal wall (including Rectus) in 60-day gestation fetal lambs, from the midline to either the right (Group A) or left (Group B) costal margin, in 14 lambs carried by 7 ewes. They were delivered by cesarean section at term (about 145 days). Scoliosis was evaluated by anterio-posterior X-rays, determining the Cobb angle., Results: Four fetuses in Group A and 3 in Group B survived. There were 3 successful AWD lambs Group A and 2 in Group B. One lamb in each group survived with the AWD covered with a thick capsule. The convexity of spinal curve was the direction of scoliosis. Right scoliosis was only seen in the 4 Group A lambs. Left scoliosis was only seen in Group B lambs (2/3, 67%). The mean Cobb angle was 41.7 ± 11.5° in Group A and in Group B the Cobb angles were 59.6 o and 60.6°. Overall, 4/5 lambs with organ prolapse (80%) and both lambs without organ prolapse had scoliosis., Conclusion: Muscle imbalance may contribute to the development of scoliosis in a fetal lamb AWD model., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Fertility preservation (FP) for children is still challenging due to an information gap. In particular, there is little information about the surgical aspects of ovarian tissue cryopreservation (OTC) for children. In the present study, the appropriateness of preoperative management and the criteria of our cases were investigated with the aim of establishing a safe OTC procedure., Methods: A total of 25 girls who underwent OTC from November 2015 through May 2020 were retrospectively analyzed with IRB approval., Results: The median age of the patients was 13 (1-17) years. The medical indications were varied (e.g., leukemia, lymphoma, brain tumor), and included rare diseases. Seventeen cases (68%) underwent OTC during chemotherapy or radiotherapy, and 21 (84%) had comorbidities. All cases underwent ovarian tissue retrieval (OTR) with laparoscopy, and the median operating time was 64 (36-97) min, with little bleeding. Although two had complications, all patients started treatment on schedule. The median WBC and CRP increases a day after OTR were 0 (- 4400 to + 5200)/µl and 0.21 (- 0.2 to 0.87) mg/dl, respectively, with no complications., Conclusion: As long as the preoperative criteria are met, OTC could be possible even for children with a severe blood condition. In such cases, the degrees of the WBC and CRP elevations are useful to assess surgical infection.

Background: We have reviewed the surgical procedures performed and outcomes for low-birth-weight infants with acquired subglottic stenosis., Methods: The gestational age at birth, birth weight, age at Laryngotracheal reconstruction, and therapeutic outcome over the past 26 years were reviewed. Laryngotracheal reconstruction was initially performed by costochondral grafting involving only the anterior wall for the first operation, but since 2010 a costal cartilage was also inserted into the posterior wall of the cricoid cartilage using the BENSON pylorus spreader to split the cartilage., Results: There were 21 patients. The mean gestational age was 29.6 weeks, the mean birth weight was 1127 g, and the first surgery was performed at a mean age of 37.0 ± 21.8 months. Extubation was possible in 11 of 21 patients (52.3%) after the first surgery and in 7 of 7 patients after re-operation. The total extubation rate reached 100% (18/18) excluding three patients (one who suffered sudden death, and two who were lost to follow-up)., Conclusion: Sufficient dilatation of the subglottic space could not be achieved by costochondral grafting involving the anterior wall alone. The extubation rate was improved by dilatation of the posterior wall and the insertion of costal cartilage into both the anterior and posterior walls.

Background: The standard surgical procedure for congenital biliary dilatation (CBD) consists of excision of extrahepatic bile duct and Roux-en-Y hepaticojejunostomy (HJ). However, alternative reconstructive operations for CBD includes hepaticoduodenostomy (HD). We compared postoperative complications and therapeutic outcomes of these two operations at our institution., Methods: From 1981 to 2009, there were 23 traceable patients who underwent operation for CBD, They were divided into an HJ Group (n = 15) and an HD Group (n = 8). Demographic and outcome data were compared., Results: There were no significant differences in postoperative complications (cholangitis, pancreatitis, and anastomotic stenosis) and current blood test data (AST, ALT, Total Bilirubin, Direct Bilirubin, Amylase) between the two groups. Current abdominal pain and carcinogenesis were not observed in either group. Intrahepatic stones occurred in one patient in each group, both > 25 years post-operation., Conclusion: HD is considered to be an acceptable reconstruction method compared to HJ, based on our results. There has been a suggestion that inadequate diversion in HD might increase the risk of cholangiocarcinoma. To date, in this series, that has not happened.

Background: We investigated the risk factors influencing ascending testis following laparoscopic percutaneous extraperitoneal closure (LPEC) for inguinal hernia or hydrocele., Methods: Boys undergoing LPEC between 2014 and 2018 had their medical records and operative movies reviewed. Group A patients required orchiopexy after LPEC. Group B patients did not. Their baseline characteristics were reviewed. The path of the LPEC needle (not crossing the spermatic duct at first circuit [Not Crossing]), whether the second entry of the LPEC needle was different from the first hole (Different Hole), peritoneal injury requiring re-ligation (Re-ligation), and hematoma (Hematoma) were evaluated. The quantitative factors of significant difference were set as a cut-off value., Results: There were 5 patients (7 sides) in Group A and 162 patients (237 sides) in Group B. Birth weight was lower in Group A (p = 0.035). Not Crossing was 7 sides (100%) in Group A and 97 sides (41%) in Group B (p = 0.002). Hematoma was 2 sides (29%) in Group A and 11 sides (5%) in Group B (p = 0.047). Cut-off value of birth weight was 932 g (AUC 0.78)., Conclusion: Birth weight < 932 g and operative findings (not crossing over the spermatic duct on the first circuit and hematoma) indicated an increased risk of ascending testis after LPEC.

Purpose: We report effects of treatment for pectus excavatum (PE) with a Vacuum Bell (VB)., Materials and Methods: Fifteen patients (13 males and 2 females) undergoing VB therapy over 6 years, aged 6-17 years (mean: 11.1 years), were divided into two Groups: Group 1 (G1), preteenagers (< 13 years); Group 2 (G2), teenagers (≧ 13 years). We retrospectively recorded changes in depth of depression and the Haller index., Results: The depth of depression reduced in 93.3% of 15 patients (mean; 8.7 mm). Minimal change occurred in the Haller index but the subcutaneous fat thickened significantly (11/15 patients). The improvement rate on elevation of the chest wall was better in G1 than G2 (G1: 54.0% vs G2: 51.3%)., Conclusion: The maximum depth of depression improved in PE patients as a result of thickening of subcutaneous fat. VB is most effective in preteenagers with PE.

Background: In congenital diaphragmatic hernia (CDH), there is pulmonary hypoplasia (PH) and also pulmonary vascular and bronchial abnormalities. Few studies have investigated bronchial maldevelopment in CDH. We evaluated bronchial area (BA) by bronchography in a fetal lamb DH model to develop a measure of PH., Methods: We created DH in fetal lambs at 75 days gestation, delivering by cesarean section and killing them at term (DH, n = 12). Normal term fetuses provided controls (C, n  = 5). We measured total lung volume (TLV) and performed barium bronchography. Using image analysis, BA, total lung area (TLA) and bronchial area/lung area ratio (B/L ratio) were calculated. Student's T test (p < 0.05; significant) and Spearman's correlation coefficient were performed., Results: TLV (ml) was 133.3 ± 41.2 in DH and 326 ± 22.5 in C (p = 0.0000001). TLA (cm 2 ) was 78.8 ± 17.4 in DH and 107.1 ± 10.3 in C (p = 0.006). BA (cm 2 ) was 39.6 ± 11.9 in DH and 52.2 ± 7.7 in C (p = 0.019). The B/L ratio was 0.45 ± 0.06 in DH and 0.49 ± 0.05 in C (p = 0.28). There are correlations in DH between TLV and TLA (r = 0.79), TLV and BA (r = 0.73) and in C between TLV and TLA (r = 0.97) and TLV and BA (r = 0.67)., Conclusion: It may be possible to assess PH on fetal MRI, given the correlation between TLV and TLA, and TLV and BA.

Background: We investigated the efficacy of broad-spectrum antibiotics for prevention of postoperative intra-abdominal abscess in pediatric acute appendicitis with our 3 risk factors:-WBC > 16.5 (× 10 3 /µl), CRP > 3.1 (mg/dl) and appendix maximum short diameter on diagnostic imaging > 11.4 mm., Methods: Four hundred twenty-two patients were reviewed. Patients with 0-1 risk factors were assessed as low-risk and those with 2-3 were high-risk. In the low-risk group, Group A (n = 66) patients received broad-spectrum antibiotics and Group B patients (n = 265) received narrow-spectrum monotherapy. In the high-risk group, Group C patients (n = 63) received broad-spectrum antibiotics and Group D patients (n = 28), narrow-spectrum antibiotics. The outcomes were the incidence of postoperative abscess and the total duration of intravenous (IV) antibiotics., Results: The incidence of intra-abdominal abscess was 6.06% in Group A versus 1.89% in Group B (p = 0.08), and 19.05% in Group C versus 3.57% in Group D (p = 0.06). Total IV antibiotic duration (days) were 6.12 ± 2.87 in Group A versus 3.83 ± 0.69 in Group B (p < 0.01), and 7.84 ± 4.57 in Group C versus 4.00 ± 0.82 in Group D (p < 0.01)., Conclusion: Broad-spectrum antibiotics did not prevent postoperative intra-abdominal abscess in either low or high-risk groups.

Background: There is no good prognostic indicator for biliary atresia (BA). We reviewed liver biopsies taken during the initial procedure to find a prognostic marker., Methods: Thirty-two BA cases underwent Kasai operation from 1976 to 2009. We compared two groups at 1, 3, and 9 years. Group A required liver transplantation or died. Group B survived with their native liver. Biopsies were analyzed for liver fibrosis, portal-central vein bridging (P-C bridging), ductal plate malformation (DPM) and the number of the bile ducts in portal canal/measured surface area of the portal canal (BDP ratio). Statistical comparisons of the multiple data were evaluated by Mann-Whitney U test, Student's t test and Pearson's Chi-square test. Regression analysis with P < 0.05 was considered significant., Results: BDP ratios (/mm(2)) were 2.4 ± 1.5 in Group A1 (n = 9) vs 4.6 ± 2.4 in Group B1 (n = 23) (P = 0.01); 2.6 ± 1.4 in Group A3 (n = 14) vs 5.1 ± 2.5 in Group B3 (n = 18) (P < 0.01), 3.0 ± 2.2 in Group A9 (n = 15) vs 4.9 ± 2.5 in Group B9 (n = 15) (P < 0.05). There was no significant difference in any other finding., Conclusion: The BDP ratio is a sound prognostic indicator in BA.

Purpose: To investigate the efficacy of our treatment of infantile hemangioma (IH)s retrospectively presenting as disfiguring or functionally threatening lesions., Materials and Methods: 25 infants with IH treated with long-pulsed dye laser or laser with or without propranolol. Tumor fading after treatment was evaluated using both color and size rating scales, before, during and after treatment., Results: 6 infants were treated by laser, 11, oral propranolol and 8 using both treatments. Each treatment was effective for most IH with a median treatment duration of 7.7 months for laser, and 13.3 months for the latter two. Combination therapy was the most effective in both color-fading and size reduction by the 3rd month. Laser alone was most effective in color-fading with almost complete disappearance of most tumors. Oral propranolol to the three site-specific IHs was effective with disappearance of tumors. There were no apparent side effects related to laser treatment, but one infant suffered from allergy and another from bronchial asthma due to propranolol medication., Conclusions: Most IHs discolored and regressed considerably with these treatments, including complete disappearance of the three functionally threatening lesions. This combined approach is quite useful for accelerating tumor regression.

Purpose: To clarify the predictors of post-operative complications of pediatric acute appendicitis., Methods: The medical records of 485 patients with acute appendicitis operated on between January 2006 and November 2014 were retrospectively reviewed. Age, sex, preoperative WBC, CRP, and appendix maximum short diameter on diagnostic imaging (AMSD) were compared retrospectively with the complications group (Group C) vs the non-complication group (Group NC) by Student's T test, Fisher exact test and Multivariate analysis. Regression analysis with p less than 0.01 was considered significant. We analyzed the most recent 314 laparoscopic appendectomy patients similarly., Results: Complications were found in 29 of the 485 appendectomies (6.0%). Comparing Group C to Group NC, preoperative WBC (×10(3)/μl) 16.4 ± 5.6 vs 14.1 ± 4.1 (p < 0.01), CRP (mg/dl) 8.3 ± 7.1 vs 3.3 ± 4.6 (p < 0.01), AMSD (mm) was 12.1 ± 3.7 vs 9.9 ± 2.8 (p < 0.01). The CRP was significantly different by Multivariate analysis, but the WBC and AMSD wasn't. The results following laparoscopic appendicectomy data were identical., Conclusion: Preoperative WBC, CPR and AMSD all indicated an increased risk of complications. If WBC (/μl) >16,500, CRP >3.1 mg/dl and AMSD >11.4 mm, complications increased sixfold.

Background: Optimal therapy for urachal remnant (UR) in children is controversial. Nonoperative management for symptomatic UR is an alternative. Many papers support the laparoscopic approach but the indication for this is unclear. We review our experience to determine the optimal management of UR., Materials and Methods: A retrospective chart review of patients from 1990 to 2013 with UR was performed. Patients were analyzed according to age, gender, initial symptoms, type of UR, treatment, and outcome., Results: We identified 27 patients (M:F = 17:10). A urachal sinus was found in 16 cases (59%), a urachal cyst in 5 (18%) and a urachal duct in 6 (22%). Eleven (A) were under 1 year, with 16 over 1 year (B). In Group A, the commonest symptom was umbilical granulation (n = 6, 54%). Group B was dominated by abdominal pain (n = 12, 75%). Six cases in Group A needed operation for repeated infections. In 5 cases, the UR disappeared. In Group B, 2 cases were followed conservatively. The others required surgery. From 2009, we utilized a laparoscopic approach (LA, n = 7) rather than the classical umbilical approach (UA, n = 13). The operation time was not significantly different (LA = 124 min: UA = 110 min, P > 0.05). There was a tendency for shorter hospital stay following LA (LA = 7.5 days: UA = 10.9 days). Complete resection was always possible using UA in Group A but a more caudal incision (mean 3.6 cm) was required in group B. LA enabled confirmation of the complete resection with three 5 mm ports in Group B. There were no operative complications after LA against two wound infections, one of which suffered a disruption, after UA., Conclusion: Conservative follow-up is recommended for UR under 1-year old except when there are repeated infections. The umbilical approach is enough for infants. Laparoscopic surgery is recommended in older children.

Background: Adriamycin mouse model (AMM) is a model of VACTERL anomalies. Sonic hedgehog (Shh) pathway, sourced by the notochord, is implicated of anorectal malformations. We hypothesized hindgut anomalies observed in the AMM are the result of abnormal effect of the notochord., Methods: Time-mated CBA/Ca mice received two intraperitoneal injections of Adriamycin (6 mg/kg) or saline as control on embryonic day (E) 7 and 8. Fetuses were harvested from E9 to E11, stained following whole mount in situ hybridization with labeled RNA probes to detect Shh and Fork head box F1(Foxf1) transcripts. Immunolocalization with endoderm marker Hnf3β was used to visualize morphology. Embryos were scanned by OPT to obtain 3D representations of expressions., Results: In AMM, the notochord was abnormally displaced ventrally with attachment to the hindgut endoderm in 71 % of the specimens. In 32 % of the treated embryos abnormal hindgut ended blindly in a cystic structure, and both of types were remarked in 29 % of treated embryos. Endodermal Shh and mesenchymal Foxf1 genes expression were preserved around the hindgut cystic malformation., Conclusions: The delamination of the developing notochord in the AMM is disrupted, which may influence signaling mechanisms from the notochord to the hindgut resulting in abnormal patterning of the hindgut.

Aim: Mucosal prolapse is a common complication following anorectoplasty for anorectal malformation. The symptoms such as soiling, staining, and pain significantly reduce the patients' quality of life. Millard et al. (Plast Reconst Surg 69(3):399-411, 1982) reported the two-flap anoplasty that creates an anal canal using two perineal pedicle skin flaps to form a "deep anus". We have used this procedure for mucosal prolapse since 1990. This study evaluated the long-term benefits of this method., Methods: From 1990 to 2009, 18 patients suffering mucosal prolapse following anorectoplasty for high imperforate anus were treated using a two-flap anoplasty (TFARP) or just mucosal resection (MR). For each procedure, the long-term clinical follow-up (maximum of 20 years) was assessed by review of medical records against the frequency of recurrence, and the recurrence of preoperative symptoms postoperatively., Results: Of the 18 patients, 8 presented with simple mucosal prolapse, 4 with bleeding, 3 with staining, 2 with incontinence, and 1 with pain. TFARP was performed for 14 patients and MR for 6 patients. In the MR group, during the maximum of 15 years follow-up, two patients (33 %) suffered a recurrence or failed to improve their symptoms such as bleeding and/or soiling. In the TFARP group, during the maximum of 20 years follow-up, there were no recurrences and their preoperative symptoms resolved completely. Furthermore, two patients in the TFARP group gained normal sensation prior to defecation., Conclusion: The advantages of this procedure were no recurrences and complete resolution of preoperative symptoms. Moreover, it provides the possibility of gaining sensate defecation, possibly because the skin flap around the anus might help develop sensation.

Intractable aspiration is a life-threatening medical problem in patients with severe motor and intellectual disabilities (SMID). Laryngotracheal separation (LTS) is a surgical procedure for the treatment of intractable aspiration which separates the upper respiratory tract from the digestive tract. We performed LTS for 14 patients with SMID to prevent intractable aspiration, performing two types of operation. The standard diversion procedure connected the upper trachea to the esophagus. The modified diversion includes closure of the proximal trachea and a high tracheostomy, avoiding a tracheoesophageal anastomosis. LTS was performed on 14 patients. Operations performed before the LTS included tracheostomy in four patients, fundoplication in six and gastrostomy in two. A standard diversion was performed in 11 patients and a modified diversion in 3. There were no operative complications. Eleven patients were safely transferred to home-care after their LTS. Twelve patients are still alive and two died some months after operation. One patient died from their primary disease and the other died a tracheo-innominate artery fistula (TIAF). We recently experienced a patient who was at high risk of developing a TIAF. LTS is an effective operation, preventing intractable aspiration in patients with severe motor and intellectual disabilities. The results are similar for the standard or modified diversion procedure with the procedure chosen being related to the initial tracheostomy site. The most serious complication is a lethal TIAF.

Cervical tumors sometimes cause airway obstruction. We have treated six children with benign cervical tumors who required tracheostomy. Two cervical and one glossal lymphangiomata treated with local injection of OK432 after creating a tracheostomy were successfully decannulated after the treatment. One patient with a giant cervical lymphangioma needed an EXIT (ex utero intrapartum treatment) procedure. He underwent tracheostomy at 10 months of age after long-term endotracheal intubation, but he died of sepsis and hypoxic brain damage at 18 months. One patient with a subglottic hemangioma treated with steroids finally achieved closure of the tracheostomy at 2 years of age. A 7-year-old girl with a tracheal schwannoma underwent tracheostomy performed a week after admission, but she already had hypoxic brain damage resulting from problems with intubation. Most patients with a lymphangioma or hemangioma in the cervical region have required early tracheostomy before commencing treatment with OK-432 or steroids. If there is any sign of possible airway compromise, then it is vital to perform an early tracheostomy, even for benign tumors.