Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy.

We present a 37 years' experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE). A retrospective territory-wide study from 1980 to 2017 on 231 patients with open KPE from three tertiary paediatric surgical centres was performed. Outcome parameters were clearance of jaundice (COJ), native liver survival (NLS) and long-term complications. Factors affecting the operative outcomes were analyzed. The median duration of follow up was 17.5 (IQR: 13.5-22) years. Over 66% of patients became jaundice-freed at 1 year after KPE. Seventy patients (30.3%) received liver transplant (LT) at a median age of 6.2 (IQR: 4.3-8.4) years. The NLS rates at 10 and 20 years were 70.7% and 61.5% respectively with no significant change over the study period. The median age at KPE was 59 (IQR: 49-67) days. KPE performed before 70 days was associated with higher odd ratios for successful drainage but the age of KPE did not have an impact on the long-term NLS. Among all native liver survivors (n = 153), the median bilirubin level was 24 (IQR: 16-36) µmol/L. Portal hypertension (PHT) and recurrent cholangitis were found in 51.6% and 27.5% of them respectively. With a vigilant follow up program, more than 60% of BA patients could remain stable with the disease and achieve long-term survival without LT. Although cholestasis, portal hypertension and recurrent cholangitis are common in long-term NLS, with a comprehensive follow management strategy, they do not always necessitate LT. Our study serves as an example for countries where deceased donor organs are scarce due to very low donation rate.

The miR-223/nuclear factor I-A axis regulates inflammation and cellular functions in intestinal tissues with necrotizing enterocolitis.

We previously demonstrated that microRNA(miR)-223 is overexpressed in intestinal tissue of infants with necrotizing enterocolitis (NEC). The objective of the current study was to identify the target gene of miR-223 and to investigate the role of the miR-223/nuclear factor I-A (NFIA) axis in cellular functions that underpin the pathophysiology of NEC. The target gene of miR-223 was identified by in silico target prediction bioinformatics, luciferase assay, and western blotting. We investigated downstream signals of miR-223 and cellular functions by overexpressing the miRNA in Caco-2 and FHs74 cells stimulated with lipopolysaccharide or lipoteichoic acid (LTA). NFIA was identified as a target gene of miR-223. Overexpression of miR-223 significantly induced MYOM1 and inhibited NFIA and RGN in Caco-2 cells, while costimulation with LTA decreased expression of GNA11, MYLK, and PRKCZ. Expression levels of GNA11, MYLK, IL-6, and IL-8 were increased, and levels of NFIA and RGN were decreased in FHs74 cells. These potential downstream genes were significantly correlated with levels of miR-223 or NFIA in primary NEC tissues. Overexpression of miR-223 significantly increased apoptosis of Caco-2 and FHs74 cells, while proliferation of FHs74 was inhibited. These results suggest that upon binding with NFIA, miR-223 regulates functional effectors in pathways of apoptosis, cell proliferation, G protein signaling, inflammation, and smooth muscle contraction. The miR-223/NFIA axis may play an important role in the pathophysiology of NEC by enhancing inflammation and tissue damage.

Impact of Age of Patient and Experience of Surgeon on the Outcome after Kasai Portoenterostomy: Can We Delay the Surgery?

INTRODUCTION: Age of patient and experience of biliary atresia (BA) center are well-known factors associated with early jaundice clearance (EJC) after Kasai portoenterostomy (KPE) in infants with BA. This study focused on the impact of age and surgeon factor on the short-term outcome after KPE within a single center. MATERIALS AND METHODS: Fifty-four consecutive infants (18 boys and 36 girls) who underwent KPE from January 2010 to January 2020 were reviewed. KPE was performed in the earliest available operative session once the initial work-up was completed. In group A (n = 41), KPE was performed by surgeon A. In group B (n = 13), KPE was performed by specialists under the supervision of surgeon B (who is the mentor of surgeon A) when surgeon A was not available for operation. The demographics of patients, the EJC (total bilirubin <20 µmol/L within 6 months of KPE), and 2-year native liver survival (NLS) between the two groups were studied. RESULTS: The median age at operation was 52 days (range 26-135 days). The overall EJC rate and 2-year NLS were 85.2 and 89.4%, respectively. Group A (p = 0.015) and male gender (p = 0.029) were statistically associated with EJC but not the age at operation (p = 0.101). Group A was also statistically associated with superior 2-year NLS (p = 0.047). CONCLUSION: Balancing between the impact of age at operation and the experience of surgeon on the outcome after KPE, our result suggested that KPE may be deferred until a more experienced surgeon to operate.

Long-Term Results of One-Stage Laparoscopic-Assisted Endorectal Pull-Through for Rectosigmoid Hirschsprung's Disease in Patients Aged Above 5 Years.

Purpose: Despite the fact that the laparoscopic-assisted endorectal pull-through (LAEPT) for Hirschsprung's disease (HD) was introduced over 20 years ago, published outcomes in older children and adolescent are lacking. To address this, we studied the long-term results of LATEP for rectosigmoid HD in patients above 5 years of age. Materials and Methods: A retrospective review was conducted on all nonsyndromic patients above 5 years of age and who underwent one-stage LAEPT for rectosigmoid HD between January 2002 and December 2017. Late Hirschsprung-associated enterocolitis (HAEC) was defined as HAEC occurring 1 year after the pull-through operation. Postoperative bowel function was assessed using the Krickenbeck classification. Results: Forty-one patients (37 males and 4 females) were included in this study with a median follow-up of 9.0 years. The median age at surgery was 55 days. Two patients had anastomotic leakage. No patient had late HAEC, rectal prolapse, anastomotic stricture, or intestinal obstruction. According to the Krickenbeck classification, all patients had voluntary bowel movements without constipation. Overall, 65.8% of patients had no soiling. However, on subgroup analysis, only 45.4% of patients younger than 11 years of age had no soiling compared with 89.5% in patients at or older than 11 years of age (P = .003). Conclusions: Our results showed that LAEPT for rectosigmoid HD was a safe procedure. Nearly 90% of patients had normal bowel function by puberty. Further studies are needed to address the problem of soiling in patients younger than 11 years of age.

Comparison of Characteristics and Outcomes in Antenatally and Postnatally Detected Choledochal Cyst in Infants and Young Children in a Laparoscopic Surgery Center.

Purpose: The aim of this study was to compare the characteristics and the outcome between infants and young children with antenatally (AN) and postnatally (PN) detected choledochal cyst (CC) in a laparoscopic surgery center. Methods: A retrospective review was conducted for all children who underwent excision of CC and hepaticojejunostomy (HJ) before 36 months of age between October 2004 and October 2019. Results: Thirty-nine children (28 girls and 11 boys) were included in this study. Twenty-one children had AN detected CC and 18 had PN detected CC. The median age at operation (AN vs. PN; 3 months vs. 15.5 months, P < .001) and body weight (AN vs. PN; 5.6 kg vs. 10.5 kg, P < .001) were significantly different between the two groups. Children in PN group has an increased risk of being symptomatic (AN vs. PN; 6 vs. 18, P < .001) and having intervention before operation (AN vs. PN; 0 vs. 4, P = .037). Laparoscopic excision was performed in all children in AN group and in 12 children (66.7%) in PN group (P = .006). Conversion to open HJ was performed in 4 children in AN group but none in PN group (P = .146). There was no statistical differences in success in laparoscopic operation (P = .257), median operative time (P = .094), postoperative complication (P = .576), and median length of hospital stay (P = .749). Conclusions: Despite younger age at operation, the outcome of laparoscopic excision of AN detected CC was comparable with PN detected CC. Earlier detection and operation decreased the risk of preoperative intervention.

Dysregulation of miR-431 and target gene FOXA1 in intestinal tissues of infants with necrotizing enterocolitis.

The level of microRNA (miR)-431 was found to be markedly up-regulated in intestinal tissue of necrotizing enterocolitis (NEC). The objective of this study was to identify the target gene of miR-431 and to investigate the role of the miR-431-FOXA1 axis in the pathophysiology of NEC. The target gene of miR-431 was identified by in silico target prediction bioinformatics, luciferase assay, and Western blotting. Effects of miR-431 on downstream expression signals, cell proliferation, and apoptosis were investigated by overexpression in Caco-2 cells upon stimulation by LPS or lipoteichoic acid (LTA). FOXA1 was identified as the target gene of miR-431. Overexpression of miR-431 in Caco-2 cells significantly inhibited FOXA1, ESRRG, and HNF4A and activated IL-6, LGR5, NFKB2, PLA2G2A, PRKCZ, and TNF. IL-8 and - 10 were enhanced when costimulated with LPS or LTA. These potential downstream genes were also significantly dysregulated in primary NEC tissues compared with surgical-control tissues. Overexpression of miR-431 significantly decreased proliferation and increased apoptosis of Caco-2 cells. A proposed network of miR-431-FOXA1 interaction with LPS and LTA receptors demonstrates dysregulation of transcription factors, inflammatory mediators, epithelium tight junction regulators, and cell proliferation and apoptosis signals. The miR-431-FOXA1 axis could in part be responsible for the intensification of the inflammatory response in NEC tissues and contribute to the proinflammatory pathophysiology.-Wu, Y. Z., Chan, K. Y. Y., Leung, K. T., Lam, H. S., Tam, Y. H., Lee, K. H., Li, K., Ng, P. C. Dysregulation of miR-431 and target gene FOXA1 in intestinal tissues of infants with necrotizing enterocolitis.

Ten-Year Native Liver Survival Rate After Laparoscopic and Open Kasai Portoenterostomy for Biliary Atresia.

PURPOSE: Laparoscopic Kasai portoenterostomy (LKP) remains controversial in the management of infants with biliary atresia (BA). There are no data reporting the 10-year native liver survival rate after LKP. The study aims to present the 10-year native liver survival rate after LKP and complications in native liver survivors after Kasai portoenterostomy (KP). METHODS: A retrospective review was conducted for 31 consecutive infants with BA who underwent KP by day 75 of life in our institute from January 1993 to December 2007. The demographics and outcomes of patients after LKP and open KP (OKP) were compared. RESULTS: Eleven patients underwent LKP and 20 patients underwent OKP. No statistical difference was observed in the age at operation and the preoperative bilirubin level. The operative time for LKP was significantly longer than that for OKP (mean 314.5 minutes versus 271.5 minutes, P = .03). The 10-year native liver survival rate was 45% (5/11) after LKP and was 85% (17/20) after OKP (P = .03). Forty percent (2/5) of the native liver survivors in the LKP and 71% (12/17) in the OKP developed complications within 10 years after KP (P = .23). CONCLUSIONS: The 10-year native liver survival rate in patients who underwent LKP by 75 days of life was 45%. With superior 10-year liver survival rate and comparable complication rate after OKP, OKP is still the treatment of choice for BA in our institute.

From Laparoscopic Pyeloplasty to Robot-Assisted Laparoscopic Pyeloplasty in Primary and Reoperative Repairs for Ureteropelvic Junction Obstruction in Children.

PURPOSE: Robot-assisted laparoscopic pyeloplasty (RALP) and laparoscopic pyeloplasty (LP) are both minimally invasive surgical options to correct ureteropelvic junction obstruction in children. There are limited data reporting surgeons' experience of switching from LP to RALP and comparing the outcomes of the two procedures. METHODS: We conducted a retrospective study on 63 consecutive children who underwent either LP or RALP by the same surgeon in a tertiary center from January 2008 to November 2016. LP had been the standard practice until January 2014 when it was replaced by RALP in short transition. RESULTS: Thirty-seven LP and 26 RALP were successfully performed in children aged 2 months to 16 years. There was no difference between the two groups in age, body weight, laterality, clinical presentations, preoperative imagings, primary/reoperative repairs. The overall success rates were 34/37 (91.9%) and 25/26 (96.2%) for LP and RALP, respectively (P > .05). RALP was associated with shorter length of stay (3.1 days versus 4.0 days; P = .03). Surgeon-in-training participation was greater in RALP group (P < .001). There was no difference in operative time, complication rates, and analgesic requirement between the two groups. CONCLUSIONS: Surgeons proficient in LP can adapt quickly to RALP, achieving comparable outcomes. Robotic technology may facilitate training on minimally invasive pyeloplasty.

Surgical Outcome Analysis of Pneumovesicoscopic Ureteral Reimplantation and Endoscopic Dextranomer/Hyaluronic Acid Injection for Primary Vesicoureteral Reflux in Children: A Multicenter 12-Year Review.

OBJECTIVE: Vesicoureteral reflux (VUR) is a common condition associated with childhood urinary tract infection (UTI), which may lead to chronic renal failure and hypertension. Different antireflux approaches were advocated with differences in morbidity and success. The aim of this study is to review and analyze the surgical outcomes of pneumovesicoscopic ureteral reimplantation and endoscopic injection of dextranomer/hyaluronic acid (Dx/HA) in three tertiary centers. MATERIALS AND METHODS: The medical records of 215 patients (159 boys and 56 girls) for a total of 323 ureters underwent surgical interventions for primary VUR from February 2002 to August 2014 were reviewed. Data on baseline demographics, preoperative symptoms, radiological imaging studies, and postoperative outcomes were analyzed. VUR resolution was defined as when no VUR was detected by micturating cystourethrogram at 3 months or later after the intervention. Independent t-test, Mann-Whitney U test, Fisher's Exact test, and Chi-Square test were used for different parameters. All results with P value ≤.05 were regarded as statistically significant. RESULTS: The mean age at operation was 3.33 and 4.63 for reimplantation and Dx/HA injection respectively. A total of 234 ureters underwent Dx/HA injection and 92 ureters underwent pneumovesicoscopic ureteral reimplantation with mean preoperative VUR grading of 3.1 and 4.2 respectively (P = .0001). The overall VUR downgrading and resolution rates were both significantly higher in reimplantation than Dx/HA injection (97.8% versus 78.6% P = .0001 and 84.3% versus 65% P = .0011). Further subgroup analyses across the different VUR gradings showed higher downgrading and resolution rates in reimplantation group than Dx/HA injection for grade 4 (100% versus 81% P = .0147 and 82.4% versus 63% P = .0411) and grade 5 VUR (97.3% versus 50% P = .0022 and 81.6% versus 40% P = .0256). Dx/HA injection was associated with shorter operation time (41.5 minutes versus 147.5 minutes, P < .001), less postoperative analgesic usage (P = .049), and shorter hospital stay (1.06 days versus 4.44 days P < .0001). No major complications were identified in both groups. The mean follow-up time was significantly longer in reimplantation group than Dx/HA group (57.25 months versus 37.85 months, P = .002). There was no significant difference in the rate of subsequent UTI development during follow-up (P = .8). CONCLUSIONS: Both Dx/HA injection and pneumovesicoscopic ureteral reimplantation are safe and effective treatments for VUR. Reimplantation is associated with significantly higher VUR downgrading and resolution rates than Dx/HA injection especially in the higher grade VUR while Dx/HA injection has significantly shorter operation time, lower postoperative analgesic usage, and shorter hospital stay. Dx/HA injection can be considered as the first line surgical treatment especially for lower grade VUR. Pneumovesicoscopic ureteral reimplantation can be used for higher grade reflux or those who failed Dx/HA treatments.

Are all patients with short segment Hirschsprung's disease equal? A retrospective multicenter study.

BACKGROUND/PURPOSE: Short segment Hirschsprung's disease (HSCR) carries a better prognosis than long segment disease, but the definition of short is controversial. The objective of this study is to determine anatomically the extent of disease involvement that would be associated with a better functional outcome. METHODS: This is a retrospective multicenter (n = 3) study with patients (≥ 3 years) who had transanal pullthrough operation done for aganglionosis limited to the recto-sigmoid colon were reviewed. The extent of disease involvement and bowel resection was retrieved by reviewing the operative records as well as histopathological reports of the resected specimens. Clinical assessment was performed according to the criteria of a seven-itemed bowel function score (BFS) (maximum score = 20). Manometric assessment was performed with anorectal manometry. RESULTS: The study period started from 2003 to 45 patients were studied with median age at assessment = 52.0 months and operation = 3.0 months. The disease involvement was categorized into upper sigmoid-descending colon (DC) (n = 8), sigmoid colon (SC) (n = 12), upper rectum (UR) (n = 14) and lower rectum (LR) (n = 11) according to the level of normal biopsy result. There was no significant difference in the age of assessment between the four groups. The median BFSs in the DC, SC, UR and LR were 13, 15, 17 and 17, respectively (p = 0.01). Nine patients from the DC and SC groups reported soiling for more than twice per week. Sub-group analysis comparing patients with and without the entire sigmoid colon resected revealed worse functional outcomes in terms of the incidence of soiling (40.7 vs 22.2%, p = 0.05) and the BFS (14 vs 18, p = 0.04) in the former group. Anorectal manometry did not reveal any significant difference between the four groups, but a higher proportion of patients in the UR and LR groups appeared to have a normal sphincter resting pressure (DC vs SC vs UR vs LR = 62.5 vs 75.0 vs 85.7 vs 80.0%, p = 0.10). CONCLUSION: Patients with short segment HSCR are not equal at all. HSCR patients with aganglionosis limited to the rectum without the need of removing the entire sigmoid colon have a better bowel control and overall functional score. Less bowel loss and colonic dissection maybe the underlying reasons. Although future studies with a larger sample size and a longer follow-up period are required to validate the results of this study, it has provided a new insight to the current understanding of short segment disease in HSCR.

Cystic meconium peritonitis with jejunoileal atresia: Is it associated with unfavorable outcome?

AIM: To compare the outcome between patients with jejunoileal atresia (JIA) associated with cystic meconium peritonitis (CMP) and patients with isolated JIA (JIA without CMP). METHODS: A retrospective study was conducted for all neonates with JIA operated in our institute from January 2005 to January 2016. Demographics including the gestation age, sex, birth weight, age at operation, the presence of associated syndrome was recorded. Clinical outcome including the type of operation performed, operative time, the need for reoperation and mortality were studied. The demographics and the outcome between the 2 groups were compared. RESULTS: During the study period, 53 neonates had JIA underwent operation in our institute. Seventeen neonates (32%) were associated with CMP. There was no statistical difference on the demographics in the two groups. Patients with CMP had earlier operation than patients with isolated JIA (mean 1.4 d vs 3 d, P = 0.038). Primary anastomosis was performed in 16 patients (94%) with CMP and 30 patients (83%) with isolated JIA (P = 0.269). Patients with CMP had longer operation (mean 190 min vs 154 min, P = 0.004). There were no statistical difference the need for reoperation (3 vs 6, P = 0.606) and mortality (2 vs 1, P = 0.269) between the two groups. CONCLUSION: Primary intestinal anastomosis can be performed in 94% of patients with JIA associated with CMP. Although patients with CMP had longer operative time, the mortality and reoperation rates were low and were comparable to patients with isolated JIA.

Spontaneous Intestinal Perforation: The Long-Term Outcome.

Background/Purpose Evaluating the long-term outcome of spontaneous intestinal perforation (SIP). Methods We studied all patients treated for SIP at our institution between January 1, 2005 and December 31, 2014. Results Twenty-three infants (13 males) with a median gestational age of 26 (range: 23-32) weeks and a median weight of 825 (range: 560-1,965) g composed this cohort. Seventeen (74%) infants had an extremely low birth weight (ELBW); nine (39%) infants were the result of multiple pregnancies.Patent ductus arteriosus (PDA) was present in 16 (70%) infants. Cyclooxygenase inhibitors were administered in 12 (52%) infants.Ten infants (seven males, 44%) were diagnosed with intraventricular hemorrhage (IVH), which was identified in the majority (8/10) at a median of 9 (range: 1-11) days prior to the perforation.All patients presented with pneumoperitoneum and underwent a laparotomy at a median age of 9 (range: 2-16) days. Twenty-one patients had an ileal perforation. A temporary stoma was placed in 21 patients, whereas two got primary anastomosis. Two (8.7%) male infants died. During the long-term follow-up period (median 6 years), six (five males) (26%) infants developed moderate to severe disabilities in combination with cerebral palsy. No surgical complications were observed. Conclusion The most important risk factor for SIP is ELBW (75%). The distal ileum is the most frequent site of perforation (88%). Approximately 40% develop IVH most often prior to the SIP. Moderate to severe neurologic disabilities are seen in more than a quarter of the children. Disability and mortality affect mostly the male sex. Long-term risks of surgical complications are very low.

Early Reoperations after Primary Repair of Jejunoileal Atresia in Newborns.

Aim: To review nine-year experience in managing jejuno-ileal atresia (JIA) by primary resection and anastomosis and identify factors associated with reoperations. Methods: From April 2006 to May 2015, all consecutive neonates who underwent bowel resection and primary anastomosis for JIA were analyzed retrospectively. Patients with temporary enterostomy were excluded. Patient demographics, types of atresia, surgical techniques, need for reoperations, and long-term outcomes were investigated. Results: A total of forty-three neonates were included, in which nineteen (44.2%) of them were preterm and fourteen (32.6%) were of low birth weight. Thirteen patients (30.2%) had jejunal atresia whereas thirty patients (69.8%) had ileal atresia. Volvulus, intussusception and meconium peritonitis were noted in 12, 8, and13 patients, respectively. Eight patients (18.6%) had short bowel syndrome after operation. Ten patients (23.3%) required reoperations from 18 days to 4 months after the initial surgery due to anastomotic stricture (n=1), adhesive intestinal obstruction (n=1), small bowel perforation (n=2) and functional obstruction (n=6). Prematurity and low birth weight were associated with functional obstruction leading to reoperation (p=0.04 and 0.01 respectively). The overall long-term survival was 97.7%. All surviving patients achieved enteral autonomy and catch-up growth at a median follow-up of 4.7 years. Conclusion: Long-term survival of JIA after primary resection and anastomosis are excellent. However, patients have substantial risk of early reoperations to tackle intraabdominal complications.

Management of Intestinal Strictures Post Conservative Treatment of Necrotizing Enterocolitis: The Long Term Outcome.

OBJECTIVES: Evaluating the long-term outcome of the surgical management for intestinal strictures developing after necrotizing enterocolitis (NEC). PATIENTS AND METHODS: This is a retrospective study of all patients with an intestinal stricture after completion of conservative management for NEC. They were treated during the eight years period from 1st January 2008 to 31st December 2015. RESULTS: During the study period 67 infants had an operation for NEC, of which 55 had emergency surgery. The remaining twelve infants (6 males) had a stricture and were included in the study group. Their median gestational age was 35 (range 27-40) weeks and the median weight was 2180 (range 770 - 3290) g. The onset of NEC was seen at a median of 2 (range 1- 47) days. The median peak C-reactive protein (CRP) level was 73.1 (range 25.2 - 232) mg/dl. Isolated strictures were seen in 9 (75%) patients. Two-third of all strictures (n=15) were located in the colon. Surgery was done at a median of 5 (range 3 - 13) weeks after diagnosing NEC. Primary anastomosis was the procedure of choice; only one needed a temporary colostomy. This cohort had no mortality during a median follow up of 6.25 (range 0.5 - 7.6) years, whilst the overall death rate for NEC was 15 (22 %). Two fifth of the group developed a neurological / sensory impairment. CONCLUSION: One fifth of the surgical workload for NEC is related to post-NEC strictures. Most strictures are located in the colonic region. In the long-term no mortality and no surgical co-morbidities were observed.

Improved outcomes after technical modifications in tubularized incised plate urethroplasty for mid-shaft and proximal hypospadias.

PURPOSE: To investigate and compare the outcomes after tubularized incised plate (TIP) urethroplasty in mid-shaft and proximal hypospadias using a standard and a modified technique. METHODS: We conducted a retrospective study in 104 consecutive children who underwent mid-shaft or proximal TIP repairs from Jan 2007 to Sept 2015. Patients in Cohort One had dorsal dartos (DD) neourethral coverage while patients in Cohort Two had either de-epithelialized split preputial (DESP) or tunica vaginalis (TV) flap coverage. TV flap was used only when DESP flap was not sufficient to cover the neourethra. RESULTS: There were 52 patients each in Cohort One (DD, n = 52) and Cohort Two (DESP, n = 38; TV, n = 14) with no difference in ratio of mid-shaft/proximal between the two cohorts. At a median follow-up of 28 months, 36 patients (34.6 %) developed 47 complications including fistula (n = 19; 18.3 %) and neourethral dehiscence (n = 4; 3.8 %). Cohort One patients had significantly more fistula (28.8 vs 7.7 %; p = 0.005) and neourethral dehiscence (7.7 vs 0 %; p = 0.04) than Cohort Two. There was no difference between the two cohorts in the complication rates of meatal stenosis, recurrent ventral curvature and neourethral stricture. CONCLUSIONS: Both DESP and TV flap appear to be superior to DD in preventing fistula and neourethral dehiscence in non-distal TIP repairs.

Laparoscopic management of antenatally detected choledochal cyst: a 10-year review.

BACKGROUND: The aim of this review was to study the characteristics and the outcome of children who underwent laparoscopic management of the antenatally detected choledochal cyst. METHODS: A retrospective review was conducted for all children who underwent excision of antenatally detected choledochal cyst and hepaticojejunostomy from 2005 to 2015. RESULTS: Fourteen patients (11 females and 3 males) were included in this study. The mean diameter of the cyst was 5.0 cm (range 2-12 cm). The mean age at operation was 3.5 months (range 11 days to 9 months). The mean body weight was 6.0 kg (range 3.9-10.0 kg). Five patients were symptomatic before operation. Laparoscopic excision of choledochal cyst was successful in all cases. The distal end of common bile duct (CBD) was cauterized in all but two cases. Conversion to open hepaticojejunostomy was required in two children. There was no intraoperative complication. Two patients had postoperative fever. One patient had minor bile leak that resolved on conservative management. The mean operative time was 286 min (range 200-390 min). The median hospital stay was 8 days (range 6-25 days). At a median follow-up of 46 months (range 6-118 months), all patients were freed from cholangitis or intestinal obstruction. Symptomatic patients had earlier operation (mean, 1.7 vs. 4.5 months p = 0.012) and were associated with postnatal increase in cyst size (p = 0.023) but were not associated with increased risk of complication or conversion (p = 1.000). CONCLUSIONS: Laparoscopic excision of choledochal cyst and hepaticojejunostomy could be safety performed in patients with the cyst detected antenatally. The distal CBD was usually small and stenotic. Symptomatic patients had earlier operation with no increase in morbidity.

Tumor risk of children with 45,X/46,XY gonadal dysgenesis in relation to their clinical presentations: Further insights into the gonadal management.

OBJECTIVE: To investigate the risk of gonadal germ cell neoplasms (GCN) in children with 45,X/46,XY gonadal dysgenesis and its relation to the clinical presentations. METHODS: We conducted a retrospective study reviewing the clinical and gonadal features of all consecutive children with 45,X/46,XY gonadal dysgenesis who received gonadal management in a tertiary center from 1985 to 2015. Study subjects were divided into Group I(significant genitalia anomaly), Group II(female phenotype) and Group III(male phenotype). RESULTS: 21 children were studied (Group I=8; Group II=11; Group III=2). All 19 children of Group I and II eventually underwent bilateral gonadectomy. One patient of Group III underwent gonadal biopsy which showed increase in fibrous tissue in the testes without any GCN. 3/8(37.5%) and 6/11(54.5%) of patients in Group I and II respectively had either gonadoblastoma (GB) or carcinoma-in-situ (CIS) or both affecting one or both gonads. Among Group I patients, the 4 dysgenetic testes affected by CIS in 3 patients were intraabdominal (n=1), inguinal (n=1) and scrotal (n=2) in positions. Among Group II patients, 6/20 streak gonads had GB and 2/2 dysgenetic testes had GB or CIS. CONCLUSIONS: 45,X/46,XY children with significant genitalia anomaly or female phenotype are both at high risk of gonadal GCN.

Dysregulated expression of arginine metabolic enzymes in human intestinal tissues of necrotizing enterocolitis and response of CaCO2 cells to bacterial components.

The small intestine is the exclusive site of arginine synthesis in neonates. Low levels of circulating arginine have been associated with the occurrence of necrotizing enterocolitis (NEC) but the mechanism of arginine dysregulation has not been fully elucidated. We aimed to investigate (i) expressional changes of arginine synthesizing and catabolic enzymes in human intestinal tissues of NEC, spontaneous intestinal perforation (SIP) and noninflammatory surgical conditions (Surg-CTL) and to investigate the (ii) mechanisms of arginine dysregulation and enterocyte proliferation upon stimulation by bacterial components, arginine depletion, ARG1 overexpression and nitric oxide (NO) supplementation. Our results showed that expressions of arginine synthesizing enzymes ALDH18A1, ASL, ASS1, CPS1, GLS, OAT and PRODH were significantly decreased in NEC compared with Surg-CTL or SIP tissues. Catabolic enzyme ARG1 was increased (>100-fold) in NEC tissues and histologically demonstrated to be expressed by infiltrating neutrophils. No change in arginine metabolic enzymes was observed between SIP and Surg-CTL tissues. In CaCO2 cells, arginine metabolic enzymes were differentially dysregulated by lipopolysaccharide or lipoteichoic acid. Depletion of arginine reduced cell proliferation and this phenomenon could be partially rescued by NO. Overexpression of ARG1 also reduced enterocyte proliferation. We provided the first expressional profile of arginine metabolic enzymes at the tissue level of NEC. Our findings suggested that arginine homeostasis was severely disturbed and could be triggered by inflammatory responses of enterocytes and infiltrating neutrophils as well as bacterial components. Such reactions could reduce arginine and NO, resulting in mucosal damage. The benefit of arginine supplementation for NEC prophylaxis merits further clinical evaluation.

What is the role of laparoscopic surgery in intussusception?

BACKGROUND: Assessing the role of laparoscopy in the management of intussusception. METHODS: A retrospective review of children aged up to 17 years who had surgery for intussusception at this institution between 1 January 2004 and 31 December 2013. RESULTS: The cohort of 44 individuals (18 females) presented at a median age of 9 months (range 2.5 months-15.75 years) with intussusception; 36 patients had undergone a failed pneumatic reduction. Thirty-seven patients had an initial laparoscopic approach. Conversion was required in 13 individuals: inability to reduce a 'tight' intussusception in seven individuals, limited working space in four individuals, and inadequate tactile response in two individuals. Twenty-four patients (54%) had the laparoscopic approach completed. An open approach was chosen for seven individuals at a median age of 5 (range 4-11) months: three individuals had marked abdominal distension, two individuals had a pneumoperitoneum and two individuals presented with a large central mass. Together with the 13 conversions, a total of 20 patients (46%) underwent an open approach. The more distal the apex of the intussusception, the more likely open surgery was. Hospital stays for the subgroup of patients with successfully completed laparoscopic intervention (n = 24) were shorter than for the open surgery group (n = 20) with P = 0.0145, but the open procedure was used to manage the more challenging cases. The subgroup of seven infants undergoing direct open surgery were significantly younger than the remaining individuals (P = 0.0046). CONCLUSION: Laparoscopic intervention is meaningful in approximately 50% of children requiring a surgical reduction.

Use of laparoscopy as the initial surgical approach of impalpable testes: 10-year experience.

AIM: To review the experience in the management of impalpable testes using laparoscopy as the initial approach and the need for inguinal exploration. METHODS: From January 2004 to June 2014, 339 patients with undescended testes underwent operation in our institute. Fifty patients (15%) had impalpable testes. All children with impalpable testes underwent initial laparoscopy. A retrospective review was conducted on this group of patients and the outcome was analyzed. RESULTS: Forty children had unilateral impalpable testis. Ten children had bilateral impalpable testes. Thirty-one children (78%) in the unilateral group underwent subsequent inguinal exploration while 4 children (40%) in the bilateral group underwent inguinal exploration (P < 0.05). Orchidopexy was performed in 16 children (40%) in the unilateral group and 9 children (90%) in the bilateral group (P < 0.05). Regarding the 24 children with unilateral impalpable testis and underwent orchidectomy for testicular nubbin (n = 19) or atrophic testes (n = 2) or has vanishing testes (n = 3); contralateral testicular hypertrophy was noticed in 10 (41%). No intra-operative complication was encountered. Two children after staged Fowler-Stephens procedure and 1 child after inguinal orchidopexy had atrophic testes. CONCLUSION: The use of laparoscopy in children with impalpable testes is a safe procedure and can guide the need for subsequent inguinal exploration. Children with unilateral impalpable testis were associated with an increased need for inguinal exploration after laparoscopy. Orchidopexies could be performed successfully in 90% of children with bilateral impalpable testes.