Safety evaluation of a stepwise tracheostomy decannulation program in pediatric patients.

PURPOSE: In the event of failed tracheostomy decannulation, patients might have a tragic course of events. We retrospectively evaluated our stepwise tracheostomy decannulation program and examined its safety. METHODS: A 12-year retrospective study of pediatric patients was conducted. The decannulation program was performed on patients who had airway patency by laryngobronchoscopy and whose cannula could be capped during the day. A stepwise decannulation program was performed: continuous 48-h capping trial during hospitalization (Phase 1), removal of the tracheostomy tube for 48 h during hospitalization (Phase 2), and outpatient observation (Phase 3). If a persistent tracheocutaneous fistula existed, the fistula was closed by surgery (Phase 4). RESULTS: The 77 patients in the study underwent 86 trials. The age at the first time of the decannulation program was 6.5 ± 3.6 years. Sixteen trials failed (18.6%): 8 trials in Phase 1, 2 trials in Phase 2, 4 trials in Phase 3, and 2 trials in Phase 4. Most decannulation failures were due to desaturation in Phase 1/2 and dyspnea in Phase 3/4. The time to reintubation after decannulation was 15-383 days in Phase 3/4. CONCLUSIONS: Patients could fail at every phase of the program, suggesting that a stepwise decannulation program contributes to safety.

The comorbidities of recurrent inguinal hernia in children: A systematic review.

BACKGROUND: Higher risk of recurrence has been reported in pediatric inguinal hernia patients with specific comorbidities. The purpose of this systematic review was to investigate which comorbidities predispose to recurrent pediatric inguinal hernias (RPIHs). METHODS: A comprehensive search of six databases was performed, reviewing the literature to date on RPIHs and the co-occurrence of comorbidities. English-language publications were considered for inclusion. The primary surgical technique (e.g., Potts procedure or laparoscopic repair) was not considered. RESULTS: Fourteen articles published between 1967 and 2021 fulfilled the inclusion criteria and did not meet the exclusion criteria. They reported a total of 86 patients diagnosed with RPIHs with 99 comorbidities. Thirty-six percent of patients had conditions with increased intra-abdominal pressure, such as ventriculoperitoneal shunt for hydrocephalus, posterior urethral valves, bladder exstrophy, seizure disorder, asthma, using continuous positive airway pressure for respiratory distress syndrome, and gastroesophageal reflux disease. Twenty-eight percent of patients had diseases with weakness of the anterior abdominal wall, specifically mucopolysaccharidosis, giant omphalocele, Ehlers-Danlos syndrome, connective-tissue disorders, and segmental spinal dysgenesis. CONCLUSIONS: The main comorbidities of RPIHs were conditions with increased intra-abdominal pressure and weakness of the anterior abdominal wall. Although these comorbidities are rare, the risk of recurrence must be noted.

Factors influencing the development of Multicystic Dysplastic Kidney (MCDK) following urinary tract obstruction in the fetal lamb.

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.

Muscle imbalance as a cause of scoliosis: a study in a fetal lamb abdominal wall defect model.

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 59.6o 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.

The Expression of Transcription Factors in Fetal Lamb Kidney.

(1) Background: Renal development involves frequent expression and loss of transcription factors, resulting in the activation of genes. Wilms' tumor 1 (WT1), hepatocyte nuclear factor-1-beta (HNF1ß), and paired box genes 2 and 8 (Pax2 and Pax8) play an important role in renal development. With this in vivo study, we examined the period and location of expression of these factors in renal development. (2) Methods: Fetal lamb kidneys (50 days from gestation to term) and adult ewe kidneys were evaluated by hematoxylin and eosin staining. Serial sections were subjected to immunohistochemistry for WT1, HNF1ß, Pax2, and Pax8. (3) Results: Pax2, Pax8, and HNF1ß expression was observed in the ureteric bud and collecting duct epithelial cells. We observed expression of WT1 alone in metanephric mesenchymal cells, glomerular epithelial cells, and interstitial cells in the medullary rays and Pax8 and HNF1ß expression in tubular epithelial cells. WT1 was highly expressed in cells more proximal to the medulla in renal vesicles and in C- and S-shaped bodies. Pax2 was expressed in the middle and peripheral regions, and HNF1ß in cells in the region in the middle of these. (4) Conclusions: WT1 is involved in nephron development. Pax2, Pax8, and HNF1ß are involved in nephron maturation and the formation of peripheral collecting ducts from the Wolffian duct.

Surgical management of unilateral oophorectomy for ovarian tissue cryopreservation in high-risk children and adolescents with varied backgrounds.

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.

Risk factors influencing ascending testis after laparoscopic percutaneous extraperitoneal closure for pediatric inguinal hernia and hydrocele.

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.

Differences in post-operative complications after reconstruction for congenital biliary dilatation in a single institution-Roux-en-Y hepaticojejunostomy versus hepaticoduodenostomy.

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.

A new approach using image analysis to assess pulmonary hypoplasia in the fetal lamb diaphragmatic hernia model.

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 (cm2) was 78.8 ± 17.4 in DH and 107.1 ± 10.3 in C (p = 0.006). BA (cm2) 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.

The role of the ureteric bud in the development of the ovine fetal kidney.

BACKGROUND: The kidney develops from an intimate interaction between the ureteric bud and the metanephric mass. We attempted to differentially stain the derivatives of the ureteric bud and the metanephric mass in ovine fetuses. METHODS: After appropriate approval, 47 fetal lambs' kidneys at 50 (4), 60 (6), 70 (5), 80 (4), 100 (10), 110 (8), 145 (10) days' gestation (term is 140-145 days) were obtained. After confirming the pregnancy, the sheep were anesthetized, and the fetuses sacrificed. The fetal kidneys were prepared for histological examination, using immunostaining for ß-catenin, Laminin, CK34ßE12, CK7, E-cadherin, and EMA. RESULTS: In the nephrogenic zone, positive staining was only seen for ß-catenin and Laminin. Areas with linear ß-catenin expression increased with increasing gestational age, whereas cytoplasmic granular expression in the nephrogenic zone diminished. At 50 days, Laminin-positive cells appeared in the ureteric bud epithelial cells, but not in the proximal tubule epithelium. They were found only in the immature collecting duct at 60 days. CONCLUSION: We have shown that the distribution of ß-catenin and Laminin positive-stained cells initially appearing in the ureteric bud changes with gestational age. Further studies may help inform the optimal timing of fetal shunt insertion in obstructive uropathy.

The effect of the broad-spectrum antibiotics for prevention of postoperative intra-abdominal abscess in pediatric acute appendicitis.

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 (× 103/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.

A Case of Congenital Infantile Fibrosarcoma of the Bowel Presenting as a Neonatal Intussusception.

Neonatal intussusception of the intestinal tract is rare. However, most neonatal intussusceptions have an organic lead point. For the lead point to be a neoplasm is extremely rare. We report a case that presented with neonatal intussusception with a congenital infantile fibrosarcoma as the lead point. The detection of ETV6-NTRK3 gene fusion was useful, although the definitive diagnosis was achieved by a comprehensive evaluation including this gene analysis, standard histology and immunohistochemistry. Neonatal intussusception should be suspected to be caused by a neoplasm. If pathological diagnosis is difficult, molecular analysis should be utilized to diagnose congenital infantile fibrosarcoma.

Evaluation of alveolar epithelial cells in the sheep model of congenital diaphragmatic hernia: Type 1 alveolar epithelial cells and histopathological image analysis.

BACKGROUND: There are few reports comparing type 1 alveolar epithelial cell development with histopathological image analysis. We investigated these as indicators of maturity in fetal lambs' lungs in a congenital diaphragmatic hernia (CDH) model. METHODS: We created left CDH in 4 fetal lambs at 75 or 76days' gestation (Group A). Controls were 5 sham-operated lambs (Group B); both groups delivered at term. The right lower lung lobe (RLL) and left lower lobe (LLL) were sampled. Using histopathological image analysis, alveoli/air sacs count (AC), alveoli/air sacs area percentage (AP), average area (AA), total area (TA), and perimeter (PM) were determined. We also evaluated total lung volumes, radial alveolar count (RAC), and Type 1 alveolar epithelial cells ratio (AT1 ratio), which we previously reported. Regression analysis was performed, with p<0.05 considered significant. RESULTS: RLL and LLL AT1 ratio and LLL RAC in Group A were lower than in Group B. There are no significant differences demonstrated by histopathological image analysis. In Group A, the AT1 ratio in the LLL was lower than in the RLL. There were no differences between LLL and RLL in Group B. CONCLUSION: AT1 ratio was superior to the other indicators evaluating lung maturity.

Prognostic factors indicating survival with native liver after Kasai procedure for biliary atresia.

BACKGROUND: The number of the bile ducts in the portal canal/measured surface area of the portal canal (BDP ratio) indicates prognosis in biliary atresia (BA), as does an elevated cytokeratin 7 positivity percentage (PCK7). We compared these two markers. METHODS: We reviewed 32 BA cases undergoing Kasai operation from 1976 to 2016 with >5 portal canals in biopsy samples. Group I required liver transplantation or died within a year of operation (n = 8). Group II survived with their native liver (n = 24). We determined the BDP ratio (102/mm2) and PCK7 (%), subdividing patients into three groups by their age at operation: Group A ≤60 days (n = 6, 1 Group I), 60< Group B ≤90days (n = 16, 5 Group I), Group C >90 days (n = 10, 2 Group I). RESULTS: PCK7 (%) was 2.71 ± 1.87 in Group I and 4.25 ± 2.56 in Group II (p = 0.13). BDP ratio (102/mm2) was 1.19 ± 0.424 in Group I and 1.64 ± 0.534 in Group II (p = 0.04). Both markers were higher in Group C than in Group A or B (p < 0.01). CONCLUSION: The BDP ratio is a better prognostic indicator than PCK7 in BA.

Does a large abdominal wall defect affect lung growth?

BACKGROUND/PURPOSE: Respiratory distress in babies with large abdominal wall defects suggests a relationship to decreased diaphragmatic movement. We evaluated pulmonary development in a fetal lamb gastroschisis model. METHODS: We created gastroschisis in 25 fetal lambs at 60days gestation (group A). Controls were 14 nonoperated lambs. (Group B) were all delivered at term. Lung volume, histology, and type 1 (AT1)/type 2 (AT2) cell ratios (AT1 ratio) were determined. We subdivided group A, comparing lambs with a large defect and scoliosis [group A (S)] with the remainder [group A (NS)]. RESULTS: Twenty-five lambs survived (11 fetuses in group A and 14 fetuses in group B). Lung volume in group A (S) was less than in group A (NS) (p<0.05). The AT1 ratio in group A was lower than in group B (p<0.01), without any difference in radial alveolar counts (RACs) or alveolar growth, and no association between scoliosis and alveolar differentiation. CONCLUSION: Gastroschisis in a sheep model reduces the AT1 ratio but not the RAC. Severe scoliosis affects lung volume but not the AT1 ratio, suggesting reduced diaphragmatic movement in fetuses with large abdominal defects.

Relation between amount of bile ducts in portal canal and outcomes in biliary atresia.

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.

Pressure limited vesico-amniotic shunt allows normal lung growth in a fetal lamb model of obstructive uropathy.

BACKGROUND: In our fetal lamb model of lower urinary tract obstruction, a pressure limited shunt preserves bladder function and renal development. This study investigates the effects on pulmonary histology. METHODS: We created obstructive uropathy (OU) in 60-day gestation fetal lambs, ligating the urethra and urachus, and delivering them at term (130-145days). We compared pulmonary histology in 4 groups: group A, OU without shunt; group B, pressure limited shunt; group C, non-valved shunt. Shunts were inserted 3weeks post-obstruction. Group D were normal controls. RESULTS: We compared 27 fetuses: 7 fetuses in group A, 4 fetuses in group B, 2 fetuses in group C, and 14 fetuses in group D. There was no significant difference in lung volume in any group. In group A, there were some areas of atelectasis and incomplete alveolar formation histologically. The alveoli in group A lambs lungs had a predominance of type II cells, whereas group B lambs lungs were lined by type I epithelial cells and were essentially indistinguishable from controls. CONCLUSIONS: This study suggests that using a pressure-limited vesico-amniotic shunt in OU may preserve the renal tract and the lungs.

Are there reliable indicators predicting post-operative complications in acute appendicitis?

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.