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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]