RESUMEN
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.
Asunto(s)
Seno Pilonidal , Enfermedades de la Piel , Humanos , Niño , Masculino , Femenino , Seno Pilonidal/cirugía , Calidad de Vida , Estudios Retrospectivos , Endoscopía , ReoperaciónRESUMEN
BACKGROUND AND AIMS: Restitution of the extrahepatic biliary luminal epithelium in cholangiopathies is poorly understood. Prominin-1 (Prom1) is a key component of epithelial ciliary body of stem/progenitor cells. Given that intrahepatic Prom1-expressing progenitor cells undergo cholangiocyte differentiation, we hypothesized that Prom1 may promote restitution of the extrahepatic bile duct (EHBD) epithelium following injury. APPROACH AND RESULTS: Utilizing various murine biliary injury models, we identified Prom1-expressing cells in the peribiliary glands of the EHBD. These Prom1-expressing cells are progenitor cells which give rise to cholangiocytes as part of the normal maintenance of the EHBD epithelium. Following injury, these cells proliferate significantly more rapidly to re-populate the biliary luminal epithelium. Null mutation of Prom1 leads to significantly >10-fold dilated peribiliary glands following rhesus rotavirus-mediated biliary injury. Cultured organoids derived from Prom1 knockout mice are comprised of biliary progenitor cells with altered apical-basal cellular polarity, significantly fewer and shorter cilia, and decreased organoid proliferation dynamics consistent with impaired cell motility. CONCLUSIONS: We, therefore, conclude that Prom1 is involved in biliary epithelial restitution following biliary injury in part through its role in supporting cell polarity.
Asunto(s)
Conductos Biliares Extrahepáticos , Colestasis , Animales , Ratones , Antígeno AC133/genética , Hígado , Epitelio , Factores de TranscripciónRESUMEN
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.
Asunto(s)
Atresia Biliar , Infecciones por Rotavirus , Rotavirus , Animales , Ratones , Antígeno AC133/genética , Atresia Biliar/metabolismo , Fibrosis , Rotavirus/metabolismo , Células Madre/metabolismo , Factores de Transcripción , Factores de Necrosis Tumoral/metabolismo , Factores de Necrosis Tumoral/farmacologíaRESUMEN
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.
Asunto(s)
Apendicitis , Laparoscopía , Herida Quirúrgica , Humanos , Niño , Apendicectomía/métodos , Apendicitis/cirugía , Estudios Retrospectivos , Narcóticos , Laparoscopía/métodos , Resultado del Tratamiento , Enfermedad Aguda , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/cirugía , Tiempo de InternaciónRESUMEN
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.
Asunto(s)
Atresia Biliar , Quistes , Preescolar , Quistes/cirugía , Conducto Hepático Común/cirugía , Humanos , Yeyunostomía , Hepatopatías , Portoenterostomía Hepática , Estudios RetrospectivosRESUMEN
INTRODUCTION: Youth in the juvenile justice system are a vulnerable, high-risk population. While the role of pediatricians and mental health professionals in providing care for these children is well studied, the surgical needs of this population are not well understood. We sought to characterize the physical trauma and surgical subspecialty needs of this population. METHODS: A retrospective chart review was performed of all children transported under custody to a stand-alone urban children's hospital. Demographic information and inpatient and outpatient encounter data were collected and analyzed. RESULTS: Between January 2020 and March 2021, 74 patients were transported for 199 subspecialty evaluations. Sixty-nine (93%) were male, 66 (89%) identified as Black, and the median age was 16 y (range, 13-20). Of all patients, 19% had at least one documented medical condition, 43% had behavioral health history, and 73% had previous arrest. Of the 199 encounters, 137 were for physical trauma (65%). Of these, 47 (34%) were for physical trauma incurred at the time of their arrest. Sixty-three patients (85%) experienced previous physical trauma (69% blunt, 12% penetrating, and 7% both), 54% had documented head trauma, 23% had a history of self-harm, and 60% of girls had experienced sexual trauma. Of the 54 children with a previous arrest, 91% had a history of physical trauma compared to 70% who were not previously incarcerated (P = 0.03). CONCLUSIONS: Most subspecialty and emergency encounters for incarcerated children are for physical trauma, revealing an opportunity for trauma-focused care in this vulnerable population. Pediatric surgeons and emergency physicians play a major role in the care of incarcerated children.
Asunto(s)
Hospitales Pediátricos , Poblaciones Vulnerables , Adolescente , Niño , Femenino , Humanos , Masculino , Estudios RetrospectivosRESUMEN
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.
Asunto(s)
Neumotórax , Tubos Torácicos , Niño , Humanos , Neumotórax/cirugía , Recurrencia , Estudios Retrospectivos , Cirugía Torácica Asistida por Video , Resultado del TratamientoRESUMEN
BACKGROUND: Currently there are no standard of care treatment strategies for IH prevention (IHP). Dehydrated human amnion-chorion (dHACM) is a healing adjunct that elutes growth factors including several that have reduced IH in animal models. We therefore performed a double-blinded, prospective randomized controlled trial (RCT) to test the hypothesis that dHACM significantly reduces IH formation in a well-studied animal model of acute IH. MATERIAL AND METHODS: Forty 16-week-old male Sprague-Dawley rats were randomized to one of four groups: No Treatment vs. dHACM Sheet (Group A), and Saline vs. dHACM Injection (Group B). Each animal underwent a 5-cm midline laparotomy which was incompletely closed with 5-0 plain gut sutures; this was performed by a surgeon blinded to treatment group (first blind). After 28 days, the primary endpoints of IH formation and hernia size were determined by study staff blinded to treatment (second blind). Secondary endpoints included healed fascia tensile strength as determined by tensiometry, systemic and local inflammatory markers as measured by ELISA, and fascial scar collagen I/III ratios per Western blotting. RESULTS: In Group A, No Treatment developed IH at 87.5% vs. 62.5% for Sheet (P = 0.28). Hernias that formed in the Sheet group were significantly smaller (P = 0.036). In Group B, Injection and Saline yielded identical IH rates of 77.8%. Molecular characterization of fascial scar demonstrated non-inferior tensile strength, collagen I/III ratios, and inflammatory markers in dHACM-treated animals. CONCLUSIONS: dHACM sheets significantly reduced the size of IH following laparotomy when compared to no treatment.
Asunto(s)
Hernia Incisional , Amnios , Animales , Corion , Modelos Animales de Enfermedad , Humanos , Masculino , Ratas , Ratas Sprague-DawleyRESUMEN
Patients with vascular Ehlers-Danlos syndrome (vEDS) have a defect in the formation of type III collagen. This defect puts patients at risk of vascular rupture, uterine rupture, and bowel perforations. The segmental absence of intestinal musculature is a rare histopathologic finding, wherein there is a lack of a muscularis propria layer in the intestinal wall. Although typically documented in the literature in neonates or adults, it can be seen in children of other ages. This is a case report of a patient who exhibits both rare entities, which has not been described in the literature to date.
RESUMEN
Five established clearing protocols were compared with a modified and simplified method to determine an optimal clearing reagent for three-dimensionally visualizing fluorophores in the murine liver, a challenging organ to clear. We report successful clearing of whole liver lobes by modification of an established protocol (UbasM) using only Ub-1, a urea-based amino sugar reagent, in a simpler protocol that requires only a 24-h processing time. With Ub-1 alone, we observed sufficiently preserved liver tissue structure in three dimensions along with excellent preservation of fluorophore emissions from endogenous protein reporters and lipophilic tracer dyes. This streamlined technique can be used for 3D cell lineage tracing and fluoroprobe-based reporter gene expression to compare various experimental conditions.
Asunto(s)
Amino Azúcares , Colorantes Fluorescentes , Hígado/diagnóstico por imagen , Urea , Animales , Fluorescencia , RatonesRESUMEN
Cholestatic liver injury is associated with intrahepatic biliary fibrosis, which can progress to cirrhosis. Resident hepatic progenitor cells (HPCs) expressing Prominin-1 (Prom1 or CD133) become activated and participate in the expansion of cholangiocytes known as the ductular reaction. Previously, we demonstrated that in biliary atresia, Prom1(+) HPCs are present within developing fibrosis and that null mutation of Prom1 significantly abrogates fibrogenesis. Here, we hypothesized that these activated Prom1-expressing HPCs promote fibrogenesis in cholestatic liver injury. Using Prom1CreERT2-nLacZ/+ ;Rosa26Lsl-GFP/+ mice, we traced the fate of Prom1-expressing HPCs in the growth of the neonatal and adult livers and in biliary fibrosis induced by bile duct ligation (BDL). Prom1-expressing cell lineage labeling with Green Fluorescent Protein (GFP) on postnatal day 1 exhibited an expanded population as well as bipotent differentiation potential toward both hepatocytes and cholangiocytes at postnatal day 35. However, in the adult liver, they lost hepatocyte differentiation potential. Upon cholestatic liver injury, adult Prom1-expressing HPCs gave rise to both PROM1(+) and PROM1(-) cholangiocytes contributing to ductular reaction without hepatocyte or myofibroblast differentiation. RNA-sequencing analysis of GFP(+) Prom1-expressing HPC lineage revealed a persistent cholangiocyte phenotype and evidence of Transforming Growth Factor-ß pathway activation. When Prom1-expressing cells were ablated with induced Diphtheria toxin in Prom1CreERT-nLacZ/+ ;Rosa26DTA/+ mice, we observed a decrease in ductular reactions and biliary fibrosis typically present in BDL as well as decreased expression of numerous fibrogenic gene markers. Our data indicate that Prom1-expressing HPCs promote biliary fibrosis associated with activation of myofibroblasts in cholestatic liver injury.