Outcomes of pediatric pilonidal disease treatment: excision with off-midline flap reconstruction versus endoscopic pilonidal sinus treatment.

PURPOSE: Pilonidal disease (PD) is marked by chronic inflammation and frequent recurrence which can decrease quality of life. However, debate remains regarding the optimal treatment for PD in the pediatric population. This study compares two recommended treatment approaches-excision with off-midline flap reconstruction (OMF: Bascom cleft lift flap, modified Limberg flap) and minimally invasive endoscopic pilonidal sinus treatment (EPSiT). METHODS: Single-center retrospective evaluation of patients 1-21 years of age with PD who underwent either excision with OMF reconstruction or EPSiT between 10/1/2011 and 10/31/2021. Outcomes included were disease recurrence, reoperation, and wound complication rates. Comparisons were performed using Chi-square and Mann-Whitney U tests. RESULTS: 18 patients underwent excision/OMF reconstruction and 45 patients underwent EPSiT. The excision/OMF reconstruction cohort was predominantly male (44.4% vs 17.8% p = 0.028), with history of prior pilonidal infection (33.3% vs 6.7%; p = 0.006), and longer median operative time (60 min vs 17 min; p < 0.001). The excision/OMF reconstruction cohort had a higher rate of wound complications (22.2% vs 0%; p = 0.001), but lower rates of disease recurrence (5.6% vs 33.3%; p = 0.022) and reoperation (5.6% vs 31.1%; p = 0.031). CONCLUSION: In pediatric patients with PD, excision with OMF reconstruction may decrease recurrence and reoperation rates with increased operative times and wound complication rates, compared to EPSiT.

TWEAK/FN14 promotes profibrogenic pathway activation in Prominin-1-expressing hepatic progenitor cells in biliary atresia.

BACKGROUND AND AIMS: Biliary atresia (BA), a congenital cholestatic liver disease, commonly culminates in end-stage liver disease. We previously demonstrated in BA that Prominin-1 ( Prom1 )-expressing hepatic progenitor cells (HPCs) expand within regions of developing fibrosis, giving rise to cholangiocytes within biliary ductular reactions. Null mutation of Prom1 or ablation of cells expressing Prom1 significantly diminishes fibrogenesis. FN14, the receptor for TNF-like weak inducer of apoptosis (TWEAK), is expressed by HPCs. TWEAK/FN14 signaling promotes fibrosis in multiple organ systems. Therefore, we hypothesized that TWEAK/FN14 signaling mediates Prom1 -expressing HPC proliferation leading to profibrogenic ductular reactions in BA. APPROACH AND RESULTS: The experimental mouse model of BA mediated by perinatal rhesus rotavirus (RRV) infection resulted in increased co-expression of Fn14 in Prom1 -expressing HPCs within regions of ductular reactions. FN14 antagonist L524-0366 decreased ductular reactions, biliary fibrosis and periportal fibroblast activation in RRV injury. L524-0366 inhibition also demonstrated loss of downstream noncanonical NF-kB signaling expression in RRV injury. Murine HPC organoids demonstrated accelerated organoid growth and proliferation when treated with recombinant TWEAK. Increased organoid proliferation with recombinant TWEAK was lost when also treated with L524-0366. Analysis of a large publicly available RNA sequencing database of BA and normal control patients revealed significant increases in expression of PROM1 , FN14 , and genes downstream of TNF signaling and noncanonical NF-κB signaling pathways in BA infants. Infants who failed to achieve bile drainage after hepatoportoenterostomy had higher relative levels of FN14 expression. CONCLUSION: TWEAK/FN14 signaling activation in Prom1 -expressing HPCs contributes to proliferation of profibrogenic ductular reactions in BA.

Postoperative narcotic utilization in single incision laparoscopic-assisted extracorporeal appendectomy (SILEA): a single-institution retrospective review.

PURPOSE: Laparoscopic appendectomy for pediatric acute appendicitis is commonly performed by pediatric surgeons. A single incision approach has been investigated, but the lack of technical uniformity has resulted in conflicting data. We hypothesized that an initial single incision laparoscopic-assisted extracorporeal appendectomy (SILEA) approach is associated with similar complication rates as compared to the standard three-incision laparoscopic appendectomy (TILA). METHODS: Approximately 1300 laparoscopic appendectomies were retrospectively reviewed for acute appendicitis over a 5 year period. Patients were split into TILA or SILEA cohorts. Propensity score matching identified 102 matched cases in both cohorts. Case and control cohort comparisons were then analyzed. RESULTS: Successful SILEA was associated with no difference in post-operative complication rate. SILEA was associated with decreased postoperative narcotic dosage and shorter operative time than TILA. CONCLUSIONS: An initial SILEA approach is safe and associated with similar complication rates as TILA. Based on this data, the authors advocate adoption of the an initial SILEA approach for uncomplicated, freely mobile, acute appendicitis with seamless conversion to TILA if the appendix is not amendable to SILEA. LEVEL OF EVIDENCE: Level III.

The Outcome of Patients With Cystic Biliary Atresia With Intact Proximal Hepatic Ducts Following Hepatic-Cyst-Jejunostomy.

OBJECTIVES: To determine the outcomes of patients with cystic biliary atresia by correlating the anatomy of the hepatic ducts with the choice of biliary reconstruction surgery. BACKGROUND: The Kasai hepatoportoenterostomy (Kasai) is the initial surgical procedure offered to most patients with biliary atresia. In contrast, a hepatic-cyst-jejunostomy has been reported to be effective in patients with the cystic form of biliary atresia. METHODS AND RESULTS: We performed an international multicenter retrospective review. Two hundred eighty-seven patients were included, and 33 cases of cystic biliary atresia were identified. Outcomes were the serum total bilirubin level 3 months post-surgery and native liver survival at 2 years of age and were compared between cases who received the Kasai versus hepatic-cyst-jejunostomy in correlation to the anatomy of proximal hepatic ducts. The patients were categorized into 3 anatomical groups: patent intact hepatic ducts (n = 10), patent hypoplastic hepatic ducts (n = 13), and obliterated hepatic ducts (n = 10). All 10 patients with patent intact hepatic duct group underwent hepatic-cyst-jejunostomy, and 9 experienced bile drainage and native liver survival. Among the 13 patients with hypoplastic hepatic ducts, 11 underwent the Kasai procedure, and 9 had bile drainage, whereas 2 underwent hepatic-cyst-jejunostomy, and one survived with the native liver. All of the patients with obliterated hepatic ducts underwent the Kasai procedure; 5 established biliary drainage and survived with the native liver. Of 5 who did not drain, 3 underwent liver transplantation. CONCLUSIONS: In patients with cystic biliary atresia, the subset with a connection between cyst and intrahepatic bile ducts via intact proximal hepatic ducts had favorable clinical outcomes following hepatic-cyst-jejunostomy.

Children with large primary spontaneous pneumothoraxes may benefit from early operative intervention.

PURPOSE: The initial management of primary spontaneous pneumothoraxes (PSP) in children remains controversial, particularly regarding the timing of operative intervention. This study aimed to identify factors associated with failure of non-operative management of PSP. METHODS: A single-center, retrospective review was performed for patients presenting with PSP. Demographics and clinical predictors were collected. Patients successfully managed non-operatively were compared to failed non-operative management. Fischer exact and Mann-Whitney tests were used as appropriate. RESULTS: Fifty-seven pediatric patients were identified as having PSP. Four patients underwent initial surgical intervention, 60% (n = 34) were successfully managed non-operatively, while 33% (n = 19) failed non-operative management and underwent video-assisted thoracic surgery (VATS). Those who failed were more likely to have PSP > 2 cm on initial X-ray (79% vs. 44%, p = 0.021) and have a persistent air leak for > 48 h (47% vs 6%, p ≤ 0.001). LOS was greater in the failure group (11.5 ± 5.1 vs 3.1 ± 2.5, p ≤ 0.001) as well as higher complication rates (21% vs 0%, p = 0.013). CONCLUSION: Our findings suggest that patients presenting with PSP of > 2 cm or have a persistent air leak for > 48 h despite chest tube management are unlikely to be treated by chest tube alone and may benefit from earlier operative intervention.