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1.

Aim

The most common cause of congenital bladder outlet obstruction (BOO) is posterior urethral valves (PUV). Initial treatment requires decompression, but transurethral incision (TUI) or primary diversion is all described. There is no randomized control trial to guide management. This study aims to describe management, circumcision, and UTI rate in a national cohort of PUV boys.

Methods

Boys diagnosed with BOO were recruited (via BAPS CASS) over 1?year with ethics committee approval (ref: 12/SC/0416). Data were collected via questionnaire, presented as number (%), analyzed by Mann–Whitney/chi-square/Fisher Exact tests, and p?<?0.05 was taken as significant.

Results

BOO presented in 121 boys during 2014–2015, and 113 were PUV. Catheter placement in 87/121(72%) was more likely to happen in antenatal vs. postnatal vs. late(> 1?y) presentations, p?<?0.0001. Polyuria occurred in 23/45(51%), 12/48(25%), 0/28(0%), respectively, p?<?0.0001. Initial surgical treatment was TUI in 108/121(89%) and vesicostomy in 2. Two ureterostomies were secondary procedures. Circumcision was performed in 52/121(43%) in antenatal presentation vs. postnatal vs. late 27/45(60%), 20/48(42%), 2/28(7%), respectively, p?=?0.01. 69 UTIs occurred in 49 patients. Circumcision was associated with an 86% reduced risk of UTI, p?<?0.0001. There was a 66% reduction in UTI risk associated with TUI alone, p?<?0.01. There was 1 death due to pulmonary hypoplasia and renal failure, and 2 experienced end-stage renal failure (ESRF).

Conclusion

Standard treatment for BOO and PUV in the current UK cohort is urethral catheterization followed by TUI. Supravesical diversion is a rescue therapy. UTIs are common and reduced by circumcision, with 43% being circumcised. Initial mortality rate was 1%, and 1.6% present in ESRF.

Level of Evidence

Prognostic study – Level I – Prospective National Cohort Study.  相似文献   

2.

Purpose

A meta-analysis including 11,900 cases showed that maternal gestational smoking was associated with increased risk of cryptorchidism. The aim of study was to investigate whether a hormone profile of cryptorchid boys and a supplementing histopathological evaluation of testicular biopsies could add detailed knowledge to the impact of maternal gestational smoking on pathogenesis of cryptorchidism.

Methods

601 cryptorchid boys aged 4?months to 14?years old were included. Because normal hormones have a pronounced age dependency, we compared results from boys whose mothers had smoked heavily (> 10 cigarettes/day) during pregnancy with age matched cryptorchid controls of nonsmoking mothers (1:6). We studied: birthweight, germ-cell number/tubular cross section, frequency of germ cells positive for placental-like alkaline phosphatase (PLAP), gonadotropins and inhibin-B.

Results

501 boys were sons of nonsmokers, 72 boys of intermittent smokers and 28 boys of heavy smokers. 39%, 44% and 61% respectively had bilateral cryptorchidism. Compared to age-matched cryptorchid controls of nonsmoking mothers, sons of heavy smokers had lower birthweight (p?=?0.006), germ-cell number/tubular cross section (p?=?0.009), frequency of germ cells positive for PLAP (p?=?0.037) and inhibin-B (p?=?0.042).

Conclusions

All findings could be associated with placental dysfunction with altered human chorionic gonadotropin production well described in women smoking during pregnancy.

Type of study

Prognosis study (prospective cohort study with > 80% follow-up).

Level of evidence

Level 1.  相似文献   

3.

Background/Purpose

The purpose of the study is to describe the anatomoclinical, diagnostic, therapeutic and prognostic aspects of pediatric follicular thyroid carcinoma (FTC) in order to choose the best therapeutic strategy.

Methods

Our study includes patients ≤ 18?years old surgically treated for FTC in four Italian Pediatric Surgery Centers from January 2000 to March 2017. The collected data were compared with those of 132 patients matched for age with a histological diagnosis of papillary thyroid carcinoma (PTC) surgically treated in the same institutions during the same period and with the data of patients diagnosed with FTC found in the literature; p-values < 0.05 were considered significant.

Results

21 (70%) of the 30 patients with a histological diagnosis of FTC underwent hemithyroidectomy while 9 (30%) underwent total thyroidectomy. 11 (55%) out of 21 patients were subjected to a completion of thyroidectomy. All patients are alive (OS?=?100%) without recurrence or relapse of the disease. Compared with PTC, FTC is significant for capsule infiltration (p?<?0.0001), vascular invasion (p?=?0.0014) and T-stage T3-T4 (p?=?0.013). However, multifocality (p?<?0.001), extrathyroid extension (p?<?0.0001) and lymph node metastasis (p?<?0.0001) are more evident in PTC.

Conclusion

The conservative approach seems to be a valid surgical treatment for pediatric patients diagnosed with MI-FTC. For patients with wide vascular invasion and/or a tumor > 4?cm, especially with high after-surgery Tg rate, a completion of thyroidectomy is recommended. In patients with multifocal neoplasia, and/or tumor size ≥ 4?cm, and/or extrathyroid extension, and/or lymph node metastasis, and/or distant metastasis, total thyroidectomy followed by radioiodine therapy is generally indicated.

Levels of Evidence

II.  相似文献   

4.

Background

Surgery for necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP) is often complicated by intestinal failure (IF) and intestinal failure associated cholestasis (IFAC).

Objective

Assessment of incidence, predictors, and mortality associated with IFAC in surgically treated NEC and SIP.Methods: A retrospective observational study based on hospital records during 1986–2014 in the two largest Finnish neonatal intensive care units was performed. IFAC was defined as conjugated bilirubin > 34?μmol/l (2.0?mg/dl) for ≥ two postoperative weeks while receiving parenteral nutrition (PN).

Results

In total 225 patients underwent surgery for NEC (n?=?142; 63%) or SIP (n?=?83; 37%). Included were 57 survivors with ≥ two weeks PN. Sixty-five (42%) patients developed IFAC. Two-year survival with IFAC was 80% and without IFAC 89% (p?=?0.13). Of the 65 patients with IFAC, all eight with unresolved IFAC died in comparison to six of 57 (11%) whose IFAC resolved (p?<?0.0001), while IFAC resolved in all survivors. Survival among patients with resolved IFAC was 89% and with unresolved IFAC (n?=?8) 0%, (p?<?0.0001). IFAC lasted for median 83 (IQR 45–120) days and correlated with the duration of PN (R2?=?0.16, p?=?0.03), delay of starting enteral feeds (R2?=?0.12, p?=?0.05) and PN lipid emulsion (RR?=?1.0 (95% CI?=?1.0–1.1) (p?=?0.02). In multivariate logistic regression analysis, IFAC development associated with septicemias and reoperations.

Conclusions

42% of prematures who underwent surgery for NEC or SIP developed IFAC. Reoperations and septicemias increased the risk of IFAC. None of the patients with unresolved IFAC survived, but IFAC did not increase overall mortality.

Type of study

Retrospective prognosis study.

Level of evidence

Level II.  相似文献   

5.

Purpose

The low perioperative mortality rate in pediatric surgery precludes effective analysis of mortality at individual institutions. Therefore, analysis of multi-institutional data is essential to determine any patterns of perioperative death in children. The aim of this study was to determine diagnoses associated with 24-hour and 30-day perioperative mortality.

Methods

A retrospective review of the 2012-2015 Pediatric Participant Use Data File (PUF) was performed. Statistical comparisons were made between survivors and nonsurvivors and between those with 24-hour and 30-day mortality using Fischer’s exact tests. P-values ≤ 0.05 were considered significant.

Results

103,444 patients who underwent a pediatric surgical operation were evaluated. There were 732 deaths with a 30-day perioperative mortality of 0.7% (732/103,444). Necrotizing enterocolitis (NEC) was the diagnosis associated with the highest 30-day perioperative mortality (175/901, 19%). A significantly higher proportion NEC deaths occurred in the first 24 hours (67% (118/175) vs 33% (57/175) 30 day mortality, p < 0.001). Compared to patients who survived following operation for NEC, those who died were statistically more likely to require inotropic support (56% vs. 15%, p < 0.001), be diagnosed with sepsis (52% vs. 22%, p < 0.001), and undergo blood transfusion within 48 hours of operation (49% vs. 34%, p < 0.001).

Conclusion

Although the overall pediatric surgical operative mortality rate is low, the largest proportion of perioperative deaths occur secondary to NEC. Based on the high immediate mortality, optimization of operative care for septic patients with NEC should be targeted.

Type of Study

Prognosis Study

Level of Evidence

Level II  相似文献   

6.

Background/purpose

Computed tomography (CT) derived Haller Index (HI) remains the standard for quantifying severity in patient with pectus excavatum (PE). Optical scanning described in literature reports optimistic results and new indices that correlate with HI. This study assessed the feasibility of a handheld White Light Scanner (WLS) to obtain 3D measurements and indices of PE deformity.

Methods

From April 2015–April 2017, WLS scanning was conducted by orthotists during clinical visits. Included were children with PE up to 18?years. Analysis assessed correlation of a WLS-derived severity index, Hebal-Malas Index (HMI), with physician measured PE Depth (PED), and CT-derived HI.

Results

Of 195 participants, 185(94%) patients with PE were scanned and 127(69%) had complete WLS data. For 88 patients undergoing monitoring, HMI correlated with PED (r?=?0.42, p?=?0.004). For 39 patients with pre-operative CT, HMI demonstrated strong correlation with HI (r?=?0.87, p < 0.0001).

Conclusions

WLS demonstrated high feasibility of scanning PE. WLS-derived HMI best correlates with HI for patients with severe pectus deformity. Our current data is suggestive that WLS is best applied for severe deformities and yet to be established for milder deformities. Future yearly WLS will provide data on deformity progression and surgical therapy.

Level of Evidence

IV.

Type of Study

Diagnostic Study.  相似文献   

7.

Purpose

To evaluate the association between Wilms tumor histology at diagnosis and the change in Wilms' tumor volume during preoperative chemotherapy.

Methods

We included all the 52 patients operated for Wilms tumor at 1988–2015, who had both pathology samples and either CT or MRI-images before and after preoperative chemotherapy, available for reevaluation.

Results

The median tumor volume was 586?ml (IQR 323–903) at diagnosis. The median change in tumor volume was ? 68% (IQR ? 85 to ? 40, p?<?0.001) and the proportion of tumor necrosis 85% (IQR 24–97), after preoperative chemotherapy. There was a correlation between blastemal cell content in prechemotherapy cutting needle biopsy (CNB) sample and the reduction in tumor volume (Rho?=?? 0.452, p?=?0.002). High stromal and epithelial cell contents in CNB samples were associated with the lesser change in tumor volume (Rho?=?0.279, p? =0.053 and Rho?=?0.300, p?=?0.038 respectively). Reduction of tumor volume and the proportion of tumor necrosis after chemotherapy were associated (Rho?=?? 0.502, p?<?0.001). The actual viable tumor volume decreased in median by 97% (IQR 65–100), and the decrease could be seen in all cellular components. In three patients, the tumor volume increased more than 10% during the preoperative chemotherapy. Two of them had anaplastic tumor in the nephrectomy specimen.

Conclusion

Wilms tumor total and viable tumor volumes were reduced by 68% and 97% with preoperative chemotherapy, respectively. High proportion of blastemal cells in CNB was associated with greatest decrease in Wilms tumor volume. Increase in tumor volume during preoperative chemotherapy may indicate anaplastic tumor and prolonging of preoperative therapy should be avoided.

Type of study

Retrospective review.

Level of evidence

Level III.  相似文献   

8.

Background/Purpose

The Abernethy malformation (AM) is a congenital venous malformation in which the splanchnic venous return bypasses the liver and drains directly into the systemic circulation. This deprives the liver of hepatotrophic growth factors and allows metabolic products of digestion to enter the systemic veins without the benefit of passing through the liver. The histologic features of liver biopsies in children with an AM were reviewed.

Methods

A retrospective review of liver biopsies in patients with AM between 1997 and 2017 was performed. Patients were divided into two groups for comparison of histologic features: presence (M +) or absence (M ?) of a coexistent liver mass on imaging. Biopsies were reviewed by a pediatric pathologist. Chi-square test was used for statistical analysis between groups. Significance was assigned to p values < 0.05.

Results

Eighteen liver biopsies were reviewed. Masses were present in only 6 patients who had a liver biopsy. Masses were observed with similar frequencies in either type of the Abernethy malformation (I or II). Nine of 12?M ? patients and 3/6?M + patients had the type I AM. Histologically, all patients were noted to have small or absent portal veins. Isolated capillaries were seen more frequently in patients with a known liver mass (p?=?0.045), while crowding of portal tracts was more commonly seen in patients without a liver mass (p?=?0.019).

Conclusion

Liver biopsies in patients with AM demonstrate abnormal vascular and parenchymal histologic features. Livers with coexistent masses were more commonly found to have features suggesting an increased dependence on arterial blood supply.

Level of evidence

III.  相似文献   

9.

Aim

We sought to assess the magnitude of functional decline and the natural history of the operated kidney residual function after zero-ischemia nephron-sparing surgery (Z-NSS) in children with unilateral renal tumor (URT).

Patients and methods

50 children were treated for URT at our surgical unit between 1992 and 2016. Of these 12 who underwent Z-NSS were available for the current analysis. Operated kidney function was assessed by 99mTc-dimercapto-succinic acid (DMSA) renal scintigraphy. Operated kidney volume was assessed by renal ultrasonography.

Results

A positive correlation between split renal function and split renal volume was found (P?=?0.001). The subset of patients with ≥ 40% preservation of operated kidney function/volume (OKF/V) had no-time dependent changes during adolescence. The subset of patients with < 40% OKF/V preservation had a catch-up growth that after puberty reached values not much different from those with ≥ 40% OKF/V preservation. At 5?years of follow-up, 3 of 5 patients with baseline dysfunction (eGFR between 40.8 and 89.4?ml/min/1.73?m2) presented with a global renal function within normal range. After puberty, all patients presented with global renal function within normal values (eGFR between 95 and 151?ml/min/1.73 m2).

Conclusions

In children with URT who underwent Z-NSS, the pattern of OKF/V recovery suggests that compensatory catch-up growth capacity during childhood minimizes OKF/V decline more than Z-NSS.

Level of evidence

Level I prognosis study — prospective cohort study with > 80% follow-up and all patients enrolled at same time point in disease.  相似文献   

10.

Background/Purpose

Massive transfusion protocols (MTPs) are considered valuable in pediatric trauma. Important questions regarding the survival benefit and optimal blood component ratio remain unknown.

Methods

The study time frame was January 2007 through December 2013 five Level I Pediatric Trauma Centers reviewed all trauma activations involving children ≤ 18?years of age. Included were patients who either had the institutional MTP or received > 20?mL/kg or?>?2?units packed red blood cells (PRBCs).

Results

110/202 qualified for inclusion. Median age was 5.9?years (3.0–11.4). 73% survived to discharge; median hospitalization was 10 (3.1–22.8) days. Survival did not vary by arrival hemoglobin (Hgb), gender or age. Partial prothrombin time (PTT), INR, GCS and injury severity score (ISS) significantly differed for nonsurvivors (all p?<?0.05). Logistic regression found increased mortality (OR 3.08 (1.10–8.57), 95% CI; p?=?0.031) per unit increase over a 1:1 ratio of pRBC:FFP.

Conclusion

In pediatric trauma pRBC:FFP ratio of 1:1 was associated with the highest survival of severely injured children receiving massive transfusion. Ratios 2:1 or ≥ 3:1 were associated with significantly increased risk of death. These data support a higher proportion of plasma products for pediatric trauma patients requiring massive transfusion.

Level of evidence

Level IV.  相似文献   

11.

Background

After NOM for BLSI, APSA guidelines recommend activity restriction for grade of injury + 2 in weeks. This study evaluates activity restriction adherence and 60?day outcomes.

Methods

Non-parametric tests and logistic regression were utilized to assess difference between adherent and non-adherent patients from a 3-year prospective study of NOM for BLSI (≤ 18?years).

Results

Of 1007 children with BLSI, 366 patients (44.1%) met the inclusion criteria of a completed 60?day follow-up; 170 (46.4%) had liver injury, 159 (43.4%) had spleen injury and 37 (10.1%) had both. Adherence to recommended activity restriction was claimed by 279 (76.3%) patients; 49 (13.4%) reported non-adherence and 38 (10.4%) patients had unknown adherence. For 279 patients who adhered to activity restrictions, unplanned return to the emergency department (ED) was noted for 35 (12.5%) with 16 (5.7%) readmitted; 202 (72.4%) returned to normal activity by 60?days. No patient bled after discharge. There was no statistical difference between adherent patients (n?=?279) and non-adherent (n?=?49) for return to ED (χ2?=?0.8 [p?<?0.4]) or readmission (χ2?=?3.0 [p?<?0.09]); for 216 high injury grade patients, there was no difference between adherent (n?=?164) and non-adherent (n?=?30) patients for return to ED (χ2?=?0.6 [p?<?0.4]) or readmission (χ2?=?1.7 [p?<?0.2]).

Conclusion

For children with BLSI, there was no difference in frequencies of bleeding or ED re-evaluation between patients adherent or non-adherent to the APSA activity restriction guideline.

Level of evidence

Level II, Prognosis.  相似文献   

12.

Background

This study compared perioperative outcomes between laparoscopic surgery (LS) and open surgery (OS) for pediatric inguinal hernia repair, using a national inpatient database.

Methods

Using the Diagnosis Procedure Combination database in Japan, we compared duration of anesthesia, postoperative complications, recurrence, and metachronous hernia (MH) between LS and OS for children undergoing inguinal hernia repair from July 2010 to March 2016. We used multivariable logistic regression analysis for postoperative complications and Cox regression analysis for recurrence.

Results

For 75,486 eligible patients (LS 20,186 vs. OS 55,300), the median follow-up was 815 (381–1350) days in LS and 1106 (576–1603) days in OS. The duration of anesthesia was significantly longer in LS than in OS for unilateral surgery (80 vs. 70 min, p < 0.001) but shorter for bilateral surgery (86 vs. 96 min, p < 0.001). LS had a lower proportion of MH than OS (0.3% vs. 3.4%, p < 0.001). There was no significant difference between LS and OS in complications (odds ratio: 0.55; 95% confidence interval: 0.22–1.38; p = 0.20) or recurrence (hazard ratio: 1.24; 95% confidence interval: 0.86–1.79; p = 0.89).

Conclusions

LS patients had lower proportions of MH than OS patients. Complications and recurrence did not differ significantly between LS and OS.

Type of study

Retrospective study.

Levels of evidence

Level III.  相似文献   

13.

Purpose

Quantify short-term outcomes associated with initial surgery [laparotomy (LAP) vs. peritoneal drain (PD)] for necrotizing enterocolitis (NEC) in extremely-low-birth-weight (ELBW) infants.

Methods

Using the Children's Hospitals Neonatal Database, we identified ELBW infants < 32?weeks' gestation with surgical NEC (sNEC). Unadjusted and multivariable regression analyses were used to estimate the associations between LAP (or PD) and death/short bowel syndrome (SBS) and length of stay (LOS).

Results

LAP was the more common initial procedure for sNEC (n?=?359/528, 68%). Infants receiving LAP were older and heavier. Initial procedure was unrelated to death/SBS in both bivariate (LAP: 43% vs PD: 46%, p?=?0.573) and multivariable analyses (OR?=?0.89, 95% CI?=?0.57, 1.38, p?=?0.6). LAP was inversely related to mortality (29% vs. 41%, p?<?0.007) in bivariate analysis, but not significant in multivariable analysis accounting for markers of preoperative illness severity. However, the association between LAP and SBS (14% vs. 5%, p?=?0.012) remained significant in multivariable analyses (adjusted OR?=?2.25, p?=?0.039). LOS among survivors was unrelated to the first surgical procedure in multivariable analysis.

Conclusion

ELBW infants who undergo LAP as the initial operative procedure for sNEC may be at higher risk for SBS without a clear in-hospital survival advantage or shorter hospitalization.

Level of evidence

Level II.  相似文献   

14.

Background

Congenital diaphragmatic hernia (CDH) is a rare birth defect with a high mortality and morbidity. Nonscrotal testes (NST) are the most reported genital anomaly in boys. Both defects have known associated anomalies, but little is known about the association between CDH and NST. This study evaluates this association and the location of the NST in a large cohort of male CDH survivors. Moreover, we analyzed possible associative factors for NST in CDH patients.

Methods

A cohort of CDH patients, born between January 2000 and March 2014 and treated in a high volume expertise center, was evaluated retrospectively. Boys with a minimum follow-up of 18 months were included. The patients were evaluated for testes location, performed orchidopexy, and possible associative factors such as birth weight, gestational age, other congenital anomalies and CDH characteristics (surgical treatment, approach and ECMO).

Results

Seventy-five CDH patients were included. Twenty-seven (36%) were diagnosed with NST, of which 22 (29%) received orchidopexy. In 54 patients (72%) there were reports on testes location at birth and location was known for all patients at the age of 18 months, although side of NST was unknown in four. The location of the NST was mostly ipsilateral to the CDH (n = 20, 87%), of which eight (35%) had a bilateral NST with a unilateral CDH. There were no significant differences in birth weight, gestational age, and CDH specific characteristics in patients with or without NST.

Conclusion

This study shows a strong association between CDH and NST, with a prevalence of 36%. However, no specific characteristics of the CDH were related to the NST. The testes of all male CDH patients should be thoroughly evaluated in the first year of their life, to ensure a proper and timely treatment.

Level of evidence

Level IV; case series.  相似文献   

15.

Objectives

Abdominal wall thickness (AWT) is a key measurement when placing or replacing low profile gastrostomy devices. This measurement varies, depending on nutritional status and body habitus. We developed a mathematical model to estimate AWT using a compendium of body measurements.

Methods

Ultrasonography was used to measure AWT at the initial gastrostomy site in subjects aged 22?days to 24?years old. Other body measurements (height, weight, waist circumference and distance from xiphisternum to pubis) were also obtained. Multiple linear regression was used to develop two separate models using age of 2?years to separate the groups. For analysis, AWT is log transformed.

Results

Data from 97 subjects were used for analysis.The final model for those ≤ 24?months old is the following:ln(Estimated AWT)?=?? 1.255?+?0.082*(1 if age 3–24?months, 0 if <3?months)?+?0.022*(waist circumference in cm).The final model for those > 24 months old is the following:ln(Estimated AWT)?=?? 1.335?+?0.271*(1 if age >84?months, 0 if 24–84 months)?+?0.082*(BMI)

Conclusion

This model to estimate AWT is useful for determining the length of a gastrostomy device at initial placement and with subsequent changes. More data are needed to refine and further validate the model.

Level of evidence

Level IV, study of prognostic test.  相似文献   

16.

Purpose

To investigate whether serial measurements of fecal calprotectin concentrations enable us to identify infants who will develop NEC prior to development of symptoms.

Methods

Prospective matched case–control study including 100 high-risk neonates. High risk includes 1) gestational age (GA) ≤ 30?weeks, 2) birth-weight (BW) ≤ 1000?g, 3) GA 30–32?weeks and BW ≤ 1250?g, 4) born from a mother who received indomethacin for tocolysis. We matched every NEC subject with three controls for birth weight and gestational age. Fecal calprotectin was measured twice a week from day one until five weeks after birth or until NEC development. We analyzed differences in fecal calprotectin between NEC subjects and controls in the week preceding NEC onset and course of fecal calprotectin within subjects who developed NEC.

Results

Of 100 included patients, ten (median GA 27.5?weeks [24.6–29.4], BW 1010?g [775–1630]) developed NEC. The median calprotectin concentration in all samples combined was 332?μg/g [< 40–8230] μg/g feces. There were no differences between NEC subjects and controls, with a wide variation in both groups. In NEC subjects, there was no intraindividual rise in calprotectin before clinical symptoms occurred.

Conclusions

There are high concentrations and wide interindividual variations in calprotectin in preterm infants during the first weeks of life. Wide intraindividual variation further precludes the serial use of fecal calprotectin in the early detection or prediction of NEC in high risk infants.

Level of Evidence

III  相似文献   

17.

Objectives

Pediatric patients born with single functional lung can be associated with symptomatic airway abnormalities. Management of such patients is not only technically demanding but is also ethically challenging. This study reports our experience of managing symptomatic airway abnormalities in pediatric patients with congenital single functional lung.

Methods

Observational retrospective cohort study performed at a tertiary children’s hospital from January 2001 to September 2017. All children (0 to 18 years old) with congenital single functional lung (agenesis and hypoplasia) presenting with symptomatic airway abnormalities (long segment congenital tracheal stenosis and tracheomalacia) and requiring surgical interventions were included in the study. Children with single functional lung secondary to non-congenital causes were excluded.

Results

A total of 16 patients with single functional lung (agenesis = 8 and hypoplasia = 8) and airway abnormalities (long segment congenital tracheal stenosis = 12 and tracheomalacia = 4) were eligible for the study. Lung abnormalities were common on the right side (n = 10, 62.5%). Associated abnormalities (cardiac and non-cardiac) were seen in 11 patients (68.8%). Surgical interventions for airway abnormalities, alone or in combination, included slide tracheoplasty (n = 12), aortopexy with or without pericardiopexy (n = 7), excision of rudimentary lung (n = 4) and placement of intrathoracic tissue expanders to reposition the mediastinum (n = 3). Nine patients (56.3%) underwent a one-stage repair while staged repairs (airway and cardiac) were performed in 7 (43.7%). Fourteen patients (87.5%) survived to hospital discharge. Of the survivors, 9 (64.2%) had stable airways not requiring respiratory support at home.

Conclusion

Management of pediatric patients with airway abnormalities in the setting of congenital single functional lung is feasible with acceptable surgical outcomes. This is facilitated by staged repairs and repositioning of mediastinum before a definitive airway repair in patients with significant comorbidities. Treatment should not be deferred to these patients if there are no obvious contraindications.

Type of Study

Retrospective Case Control Study

Level of Evidence

Level III.  相似文献   

18.

Background

Although serial transverse enteroplasty (STEP) improves function of dilated short bowel, a significant proportion of patients require repeat surgery. To address underlying reasons for unsuccessful STEP, we compared small intestinal mucosal characteristics between initial and repeat STEP procedures in children with short bowel syndrome (SBS).

Methods

Fifteen SBS children, who underwent 13 first and 7 repeat STEP procedures with full thickness small bowel samples at median age 1.5 years (IQR 0.7–3.7) were included. The specimens were analyzed histologically for mucosal morphology, inflammation and muscular thickness. Mucosal proliferation and apoptosis was analyzed with MIB1 and Tunel immunohistochemistry.

Results

Median small bowel length increased 42% by initial STEP and 13% by repeat STEP (p = 0.05), while enteral caloric intake increased from 6% to 36% (p = 0.07) during 14 (12-42) months between the procedures. Abnormal mucosal inflammation was frequently observed both at initial (69%) and additional STEP (86%, p = 0.52) surgery. Villus height, crypt depth, enterocyte proliferation and apoptosis as well as muscular thickness were comparable at first and repeat STEP (p > 0.05 for all). Patients, who required repeat STEP tended to be younger (p = 0.057) with less apoptotic crypt cells (p = 0.031) at first STEP. Absence of ileocecal valve associated with increased intraepithelial leukocyte count and reduced crypt cell proliferation index (p < 0.05 for both).

Conclusions

No adaptive mucosal hyperplasia or muscular alterations occurred between first and repeat STEP. Persistent inflammation and lacking mucosal growth may contribute to continuing bowel dysfunction in SBS children, who require repeat STEP procedure, especially after removal of the ileocecal valve.

Level of evidence

Level IV, retrospective study.  相似文献   

19.

Purpose

To assess the efficacy of combined transcutaneous interferential (IF) electrical stimulation and pelvic floor muscle training through biofeedback on non-neuropathic urinary incontinence in children.

Methods

This prospective study comprised of 46 anatomically and neurologically normal children (9 boys, 37 girls; mean age of 8.4?±?2.2?years old) with non-neuropathic urinary incontinence. All children were evaluated by kidney and bladder ultrasounds, uroflowmetry with electromyography (EMG), a complete voiding diary and a dysfunctional voiding scoring questionnaire at the baseline. Children were randomly allocated into two treatment groups including group A (n?=?23) who underwent biofeedback therapy in addition to IF electrical stimulation and group B (n?=?23) who received only biofeedback therapy. Re-evaluation was performed 6?months and one year after completion of the treatment sessions.

Results

Improvement of non-neuropathic urinary incontinence was significantly higher in group A in comparison to group B at two follow ups (P?<?0.05). Daytime incontinence was improved in 19/23(82%) and 13/23(56.5%) of children in groups A and B respectively after the treatment (P?<?0.01). There was no significant difference in uroflowmetry measures between two groups after the treatment.

Conclusions

Combination of biofeedback therapy and transcutaneous IF electrical stimulation is a potential effective modality in treating non-neuropathic urinary incontinence in children.

Level of Evidence

Type of study: Treatment study.Level I: Randomized controlled trials with adequate statistical power to detect differences (narrow confidence intervals) and follow up > 80%.  相似文献   

20.

Aim

To evaluate echocardiographic indices of pulmonary vascular resistance and right ventricular (RV) function in predicting death or ECMO in congenital diaphragmatic hernia (CDH).

Methods

In this single center study, early (< 48?h) echocardiograms of neonates with CDH (n?=?47) were reviewed by a single reader for the ratio of tricuspid regurgitant velocity to velocity time integral at the pulmonary valve (TRV/VTIpv), TRV2/VTIpv, RV fractional area change (FAC) and tricuspid annular plane systolic excursion (TAPSE). Receiver operating characteristic curves were generated for each parameter to obtain optimal cutoff values.

Results

Infants who died or received ECMO (n?=?30) had significantly higher TRV/VTIpv and TRV2/VTIpv, and lower VTIpv, RV FAC and TAPSE, compared to the 17 survivors without ECMO. For TRV2/VTIpv and TRV/VTI at cutoffs of 1.3 and 0.38 respectively, the sensitivities were 92.3 (95% CI 75–99.1%) and 96 (95% CI 80.4–99.9%), specificities were 78.6 (49.2–95.4%) and 71.4 (42–91.6%), positive predictive values were 89 (75–95.6%) and 86.2 (73.2–93.5%) and negative predictive values were 84.6 (58.5–95.5%) and 90.9 (58.7–98.6%).

Conclusion

Early echocardiographic evidence of pulmonary hypertension and RV dysfunction predicts outcome in CDH. Further studies utilizing these indices to guide therapeutic decision-making are warranted.

Level of evidence

Level 1 for diagnostic study  相似文献   

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