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OBJECTIVES: In pediatric patients with intestinal failure (IF) due to short bowel syndrome, we hypothesized that young children, those with shorter residual small bowel and those with congenital malrotation of the bowel would be more likely to undergo pan-enteroscopy. We aimed to determine the feasibility and diagnostic yield of pan-enteroscopy in this cohort. METHODS: We performed a single-center, retrospective study of pediatric patients with IF due to short bowel syndrome (SBS) who had undergone at least one GI endoscopic evaluation between January 1, 2018 and January 1, 2023. RESULTS: A pan-enteroscopy might have been possible in 381 of the 431 procedures (206 patients) reviewed. 44 (21%) patients underwent 54 pan-enteroscopies. Children with a residual bowel length <35 cm had higher odds of undergoing pan-enteroscopy (odds ratio [OR] 3.72, 95% confidence interval [CI] [1.32, 10.48], p = 0.01), as did patients with periprocedural glucagon-like peptide 2 (GLP-2) analog use (OR 4.30, 95% CI [1.24, 14.95], p = 0.02). Patients with diagnoses other than necrotizing enterocolitis (NEC) tended to be more likely to achieve a pan-enteroscopy (OR 2.73, 95% CI [0.95,7.88], p = 0.06). Evidence of gross and histopathologic abnormalities were found in 77.8% and 78% of the procedures, respectively. No complications were identified. CONCLUSION: In a large cohort of children with SBS, pan-enteroscopy was successfully performed in 14.2% of the procedures and microscopic abnormalities were common. Shorter residual bowel length, underlying diagnoses of non-NEC, and GLP-2 analog use were generally associated with successful pan-enteroscopy, independent of age and several other factors. These data suggest that pan-enteroscopy is feasible and of high-yield in a subset of patients with SBS.
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The natural history of short bowel syndrome involves intestinal adaptation wherein the remnant small intestine undergoes histologic and anatomic changes aimed at increasing absorption. Teduglutide-a glucagon-like peptide 2 analog approved for pediatric use in 2019-stimulates this process by causing proliferation of intestinal epithelial cells resulting in increased villous height and crypt depth. Food and Drug Administration approval for pediatric patients followed safety and efficacy studies in children that were limited to 24-week duration. Pediatric-specific postmarketing studies evaluating long-term safety and efficacy are underway. Formation of colorectal polyps has been repeatedly observed in studies of adult patients on long-term teduglutide, including in individuals without endoscopic evidence of polyps before treatment initiation. Recent studies, however, suggest increased risk of small bowel hyperplastic and dysplastic polyp formation with long-term glucagon-like peptide 2 analog use. We report 2 cases of small bowel foveolar hyperplastic polyps found during surveillance endoscopies after 1 year of treatment with teduglutide.
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Gastrointestinal xanthomas are benign, usually sessile, polypoid lesions occasionally incidentally seen in adults, usually in the stomach, but have not been reported in the large intestine in children. We identified xanthomas in the sigmoid colon of the 15-year-old girl confirmed histologically. Our findings suggest that colonic xanthomas may occur as an incidental finding in pediatric patients. They have a characteristic visual and histologic appearance but do not appear to be associated with any symptoms or illness and do not require follow-up.
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OBJECTIVE: The objective of this study is (1) to describe the prevalence of pancreatitis-associated medication (PAM) use at admission and discharge in pediatric and young adult patients hospitalized with acute pancreatitis (AP) and (2) to describe the prevalence of PAM use at admission in patients classified as having idiopathic AP. STUDY DESIGN: A single-center retrospective study of patients <21 years who were hospitalized with AP or acute recurrent pancreatitis from March 2015 to July 2017 was performed. Charts were reviewed for demographic data, etiology of pancreatitis, comorbidities, and use of PAMs at admission and discharge. PAMs were defined and scored based on an evidence-based classification system, with class I PAMs having strongest evidence for causation. Standard descriptive statistics were used to report prevalence data. RESULTS: Our cohort was comprised of 119 patients; 50% of patients were using a PAM at admission and 67% were taking a PAM at discharge, reflecting a significant change (P = 0.0009); 44% of patients classified as having idiopathic pancreatitis were taking a PAM on admission, reflecting a possibly missed role of medication in their presentation. Comorbidities significantly associated with PAM use included seizure disorder (P = 0.005) and oncologic disease (P = 0.005). The most commonly used class I PAMs were omeprazole, trimethoprim-sulfamethazole, valproic acid, and 6-mercaptopurine. The increase in prevalence of PAM use at discharge compared to admission was partially driven by addition of omeprazole to the outpatient medication regimen during the hospital stay (P = 0.07). CONCLUSION: Medications likely play an under-recognized role in pediatric AP. The practice of using proton pump inhibitors in management of AP warrants further study.
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Pancreatitis , Humanos , Niño , Adulto Joven , Pancreatitis/etiología , Estudios Retrospectivos , Enfermedad Aguda , Hospitalización , OmeprazolRESUMEN
Previous studies have demonstrated the safety of performing endoscopic retrograde cholangiopancreatography (ERCP) in the pediatric population; however, few have addressed the outcomes of children undergoing ERCP during acute pancreatitis (AP). We hypothesize that ERCP performed in the setting of AP can be executed with similar technical success and adverse event profiles to those in pediatric patients without pancreatitis. Using the Pediatric ERCP Database Initiative, a multi-national and multi-institutional prospectively collected dataset, we analyzed 1124 ERCPs. One hundred and ninety-four (17%) of these procedures were performed in the setting of AP. There were no difference in the procedure success rate, procedure time, cannulation time, fluoroscopy time, or American Society of Anesthesiology class despite patients with AP having higher American Society of Gastrointestinal Endoscopy grading difficulty scores. This study suggests that ERCP can be safely and efficiently performed in pediatric patients with AP when appropriately indicated.
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Colangiopancreatografia Retrógrada Endoscópica , Pancreatitis , Niño , Humanos , Colangiopancreatografia Retrógrada Endoscópica/efectos adversos , Colangiopancreatografia Retrógrada Endoscópica/métodos , Pancreatitis/diagnóstico por imagen , Pancreatitis/cirugía , Pancreatitis/epidemiología , Enfermedad Aguda , Estudios Retrospectivos , FluoroscopíaRESUMEN
This study examines the role of trainee involvement with pediatric endoscopic retrograde cholangiopancreatography (ERCP) and whether it affects the procedure's success, post-procedural adverse outcomes, and duration. A secondary analysis of the Pediatric ERCP Database Initiative, an international database, was performed. Consecutive ERCPs on children <19 years of age from 18 centers were entered prospectively into the database. In total 1124 ERCPs were entered into the database, of which 320 (28%) were performed by trainees. The results showed that the presence of trainees did not impact technical success ( P = 0.65) or adverse events rates ( P = 0.43). Rates of post-ERCP pancreatitis, pain, and bleeding were similar between groups ( P > 0.05). Fewer cases involving trainees were in the top quartile (>58 minutes) of procedural time (19% vs 26%; P = 0.02). Overall, our findings indicate trainee involvement in pediatric ERCP is safe.
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Colangiopancreatografia Retrógrada Endoscópica , Pancreatitis , Niño , Humanos , Colangiopancreatografia Retrógrada Endoscópica/efectos adversos , Colangiopancreatografia Retrógrada Endoscópica/métodos , Pancreatitis/epidemiología , Pancreatitis/etiología , Estudios RetrospectivosRESUMEN
OBJECTIVES: Endoscopic retrograde cholangiopancreatography (ERCP) in adults has been extensively studied through multicenter prospective studies. Similar pediatric studies are lacking. The Pediatric ERCP Database Initiative (PEDI) is a multicenter collaborative aiming to evaluate the indications and technical outcomes in pediatric ERCPs. METHODS: In this prospective cohort study, data were recorded for pediatric ERCPs performed across 15 centers. A pre-procedure, procedure, 2-week post-procedure follow-up, and adverse event form were completed for each ERCP performed. Univariate and stepwise linear regression was performed to identify factors associated with technically successful procedures and adverse events. RESULTS: A total of 1124 ERCPs were performed on 857 patients from May 1, 2014 to May 1, 2018. The median age was 13.5 years [interquartile range (IQR) 9.0-15.7]. Procedures were technically successful in the majority of cases (90.5%) with success more commonly encountered for procedures with biliary indications [odds ratio (OR) 4.2] and less commonly encountered for native papilla anatomy (OR 0.4) and in children <3 years (OR 0.3). Cannulation was more often successful with biliary cannulation (95.9%) compared to pancreatic cannulation via the major papilla (89.6%, P < 0.0001) or minor papilla (71.2%, P < 0.0005). The most commonly identified adverse events included post-ERCP pancreatitis (5%), pain not related to post-ERCP pancreatitis (1.8%), and bleeding (1.2%). Risk factors for the development of each were identified. CONCLUSIONS: This large prospective study demonstrates that ERCP is reliable and safe in the pediatric population. It highlights the utility of PEDI in evaluating the technical outcomes of pediatric ERCPs and demonstrates the potential of PEDI for future studies in pediatric ERCPs.
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Colangiopancreatografia Retrógrada Endoscópica , Pancreatitis , Humanos , Niño , Adulto , Adolescente , Colangiopancreatografia Retrógrada Endoscópica/efectos adversos , Colangiopancreatografia Retrógrada Endoscópica/métodos , Estudios Prospectivos , Estudios Retrospectivos , Cateterismo/efectos adversos , Pancreatitis/etiologíaRESUMEN
Serrated polyps are pathological neoplastic lesions in the colon with subtle gross morphology leading to underreporting during colonoscopy. While detection rates are increasing in average-risk adult screening colonoscopy, the rate of detection during pediatric colonoscopy is unknown. Serrated polyposis syndrome is characterized by the presence of multiple serrated polyps in the colon and an increased risk of developing colorectal cancer. Cancer prevention relies on early recognition, endoscopic clearance of all polyps > 5 mm, and continued interval surveillance or prophylactic colectomy. We report the diagnosis and management of serrated polyposis syndrome in a young adolescent patient and highlight the subtle features of serrated polyps that may go unrecognized leading to underreporting in childhood.
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Poliposis Adenomatosa del Colon , Pólipos del Colon , Neoplasias Colorrectales , Poliposis Adenomatosa del Colon/complicaciones , Poliposis Adenomatosa del Colon/diagnóstico , Poliposis Adenomatosa del Colon/cirugía , Adolescente , Adulto , Niño , Colectomía , Pólipos del Colon/diagnóstico , Pólipos del Colon/patología , Pólipos del Colon/cirugía , Colonoscopía , Neoplasias Colorrectales/diagnóstico , Humanos , SíndromeRESUMEN
INTRODUCTION: Ultrasound screening for thyroid cancer is recommended in familial adenomatous polyposis (FAP). This study investigated the prevalence of thyroid neoplasia in children with FAP. METHODS: Cross-sectional study of children with FAP at an academic hospital. Clinical and ultrasound data were analyzed for the prevalence of thyroid nodules and cancer. RESULTS: Of 37 children with FAP, 8 (22%) had thyroid nodules and 2 (5%) had thyroid cancer. Nodules (30%) and cancer (9%) were more common among female subjects and rare among male subjects. DISCUSSION: Thyroid ultrasound screening in adolescence may benefit female subjects with FAP but has limited utility in male subjects.
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Poliposis Adenomatosa del Colon , Neoplasias de la Tiroides , Nódulo Tiroideo , Poliposis Adenomatosa del Colon/diagnóstico por imagen , Poliposis Adenomatosa del Colon/epidemiología , Adolescente , Niño , Estudios Transversales , Femenino , Humanos , Masculino , Neoplasias de la Tiroides/diagnóstico por imagen , Neoplasias de la Tiroides/epidemiología , Nódulo Tiroideo/diagnóstico por imagen , Nódulo Tiroideo/epidemiologíaRESUMEN
BACKGROUND: Triple gallbladder is a rare congenital anomaly of the biliary tract that can be associated with heterotopic tissue. Gallbladder triplication results from the failure of rudimentary bile ducts to regress during embryological development, and can be difficult to distinguish from Todani type II choledochal cysts and biliary duplication cysts. CASE PRESENTATION: A 2-year-old patient presented to our institution with intermittent abdominal pain for 1 year. She had elevated transaminases with imaging concerning for a choledochal cyst. After assessment with magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography, she was diagnosed with a gallbladder multiplication and a common bile duct stricture. She underwent laparoscopic cholecystectomy, which confirmed the diagnosis of triple gallbladder. One of the three gallbladders demonstrated heterotopic gastric mucosa on final pathology, including at the cystic duct margin. Follow up testing with a technetium 99 m scan demonstrated a subtle focus of increased activity in the right upper abdomen at the expected location of the common bile duct, concerning for the presence of residual gastric mucosa. The patient remains well without abdominal pain. CONCLUSIONS: We describe the first case of heterotopic gastric mucosa in a triple gallbladder in a young patient presenting with chronic abdominal pain. We also demonstrate the safety and feasibility of laparoscopic cholecystectomy in young children with triple gallbladder. Finally, we propose an interdisciplinary approach to the management of common bile duct strictures in the setting of ectopic acid secretion, involving a combination of medical management, endoscopic intervention, and possible salvage laparoscopic Roux-en-Y hepaticojejunostomy.
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Quiste del Colédoco , Vesícula Biliar , Abdomen/patología , Niño , Preescolar , Colangiopancreatografia Retrógrada Endoscópica , Quiste del Colédoco/complicaciones , Femenino , Vesícula Biliar/diagnóstico por imagen , Vesícula Biliar/cirugía , Mucosa Gástrica/patología , HumanosRESUMEN
BACKGROUND AND AIMS: Ionizing radiation exposure during endoscopic retrograde cholangiopancreatography (ERCP) is an important quality issue especially in children. We aim to identify factors associated with extended fluoroscopy time (FT) in children undergoing ERCP. METHODS: ERCP on children <18âyears from 15 centers were entered prospectively into a REDCap database from May 2014 until May 2018. Data were retrospectively evaluated for outcome and quality measures. A univariate and step-wise linear regression analysis was performed to identify factors associated with increased FT. RESULTS: 1073 ERCPs performed in 816 unique patients met inclusion criteria. Median age was 12.2âyears (interquartile range [IQR] 9.3-15.8). 767 (71%) patients had native papillae. The median FT was 120âseconds (IQR 60-240). Factors associated with increased FT included procedures performed on patients with chronic pancreatitis, ERCPs with American Society of Gastrointestinal Endoscopy (ASGE) difficulty grade >3, ERCPs performed by pediatric gastroenterologist (GI) with adult GI supervision, and ERCPs performed at non-free standing children's hospitals. Hispanic ethnicity was the only factor associated with lower FT. CONCLUSION: Several factors were associated with prolonged FTs in pediatric ERCP that differed from adult studies. This underscores that adult quality indicators cannot always be translated to pediatric patients. This data can better identify children with higher risk for radiation exposure and improve quality outcomes during pediatric ERCP.
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Colangiopancreatografia Retrógrada Endoscópica , Exposición a la Radiación , Adulto , Niño , Colangiopancreatografia Retrógrada Endoscópica/efectos adversos , Colangiopancreatografia Retrógrada Endoscópica/métodos , Estudios de Cohortes , Fluoroscopía/efectos adversos , Humanos , Exposición a la Radiación/efectos adversos , Estudios RetrospectivosRESUMEN
Ultra-rare genetic disorders can provide proof of concept for efficacy of targeted therapeutics and reveal pathogenic mechanisms relevant to more common conditions. Juvenile polyposis of infancy (JPI) is caused by microdeletions in chromosome 10 that result in haploinsufficiency of two tumor suppressor genes: phosphatase and tensin homolog deleted on chromosome 10 (PTEN) and bone morphogenetic protein receptor type IA (BMPR1A). Loss of PTEN and BMPR1A results in a much more severe phenotype than deletion of either gene alone, with infantile onset pan-enteric polyposis and a high mortality rate. No effective pharmacological therapy exists. A multi-center cohort analysis was performed to characterize phenotype and investigate the therapeutic effect of mammalian target of rapamycin (mTOR) inhibition (adverse events, disease progression, time to colectomy and mortality) in patients with JPI. Among 25 JPI patients identified (mean age of onset 13 months), seven received mTOR inhibitors (everolimus, n = 2; or sirolimus, n = 5). Treatment with an mTOR inhibitor reduced the risk of colectomy (hazard ratio = 0.27, 95% confidence interval = 0.07-0.954, P = 0.042) and resulted in significant improvements in the serum albumin level (mean increase = 16.3 g/l, P = 0.0003) and hemoglobin (mean increase = 2.68 g/dl, P = 0.0077). Long-term mTOR inhibitor treatment was well tolerated over an accumulated follow-up time of 29.8 patient years. No serious adverse events were reported. Early therapy with mTOR inhibitors offers effective, pathway-specific and personalized treatment for patients with JPI. Inhibition of the phosphoinositol-3-kinase-AKT-mTOR pathway mitigates the detrimental synergistic effects of combined PTEN-BMPR1A deletion. This is the first effective pharmacological treatment identified for a hamartomatous polyposis syndrome.
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Inhibidores mTOR , Síndromes Neoplásicos Hereditarios , Receptores de Proteínas Morfogenéticas Óseas de Tipo 1 , Colectomía , Hemorragia Gastrointestinal , Humanos , Poliposis Intestinal/congénito , Síndromes Neoplásicos Hereditarios/genética , Síndromes Neoplásicos Hereditarios/patología , Síndromes Neoplásicos Hereditarios/cirugía , Fosfohidrolasa PTEN/genética , Serina-Treonina Quinasas TOR/genéticaRESUMEN
BACKGROUND: Propofol and dexmedetomidine are used separately, and sometimes together, for paediatric deep sedation. Although their combination has been described in adults, the effect of dexmedetomidine as a potential synergist in children has not been studied before. OBJECTIVES: The primary objective was to compare the propofol requirements of children who receive propofol alone with those who receive it in combination with dexmedetomidine for deep sedation for upper and lower endoscopic gastrointestinal procedures. DESIGN: This was a prospective, open-label, randomised study comparing patients who received propofol alone (group P) with those who received dexmedetomidine and propofol (group DP). The depth of sedation was titrated to a target bispectral (BIS) index. SETTING: A Gastroenterology Procedure Unit at a single, tertiary care academic medical centre from April 2018 until December 2019. PATIENTS: Eligible patients were scheduled for upper endoscopy, lower endoscopy or both. A total of 39 patients were enrolled (20 DP) and (19 P). INTERVENTIONS: Patients in Group DP received dexmedetomidine 0.5âµgâkg-1 administered over 1âmin followed by an infusion of 0.15âµgâkg-1âh-1. In both groups, intravenous propofol was given in bolus increments titrated to a BIS index of 40 to 50 and then a continuous infusion of propofol to maintain BIS at 40 to 50. MAIN OUTCOME MEASURES: The primary outcome measure was propofol requirement in each group. Secondary outcome measures were time to achieve the targeted sedation depth, time to achieve an Aldrete recovery score of 9, duration of sedation, mean BIS values, adverse events, 'PAED' scores and time to discharge from the postanaesthesia care unit (PACU). RESULTS: The median (range) total dose of propofol was 0.23 (0.10 to 0.50) mgâkg-1âmin-1 in group DP and 0.40 (0.20 to 0.50) mgâkg-1âmin-1 in group P (Pâ=â0.0004). Time of discharge from the PACU was 60 (20 to 121) min in group DP and 63 (46 to 91) min in group P (Pâ=â0.0409). CONCLUSION: The combination of dexmedetomidine and propofol for paediatric procedural sedation achieved a significant reduction in median propofol dose and a slightly shorter median time to discharge from PACU. Large-scale studies may determine whether this reduction decreases the risk of significant adverse events. TRIAL REGISTRATION: ClinicalTrials.gov identifier NCT02952222.
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Sedación Profunda , Dexmedetomidina , Propofol , Adulto , Niño , Dexmedetomidina/efectos adversos , Humanos , Hipnóticos y Sedantes , Estudios ProspectivosAsunto(s)
Esófago de Barrett , Niño , Unión Esofagogástrica , Estudios de Seguimiento , Humanos , MetaplasiaRESUMEN
BACKGROUND: Pancreatic pseudocysts may develop after high-grade pancreatic injuries in children. Many resolve without intervention, and the management of symptomatic pseudocysts that persist remains controversial, with various open, percutaneous and laparoscopic approaches to intervention described. Successful endoscopic cyst gastrostomy has been reported in children with pancreatic pseudocysts of mixed etiology. METHODS: The trauma registry and electronic medical record of a level 1 pediatric trauma center were queried for children with a symptomatic pseudocyst following pancreatic trauma over a 12-year period, from 2008 to 2019. RESULTS: We describe a case series of five consecutive children with persistent symptomatic pancreatic pseudocysts following blunt abdominal trauma all successfully treated with endoscopic cyst gastrostomy. DISCUSSION: Endoscopic cyst gastrostomy appears to be safe and effective in the management of symptomatic pancreatic pseudocysts in children following pancreatic trauma. LEVEL OF EVIDENCE: 5 - retrospective case series.
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OBJECTIVES: Barrett esophagus (BE) and intestinal metaplasia of gastroesophageal junction (IMGEJ) are rare in the pediatric population. This multi-institutional retrospective study evaluated the clinicopathologic characteristics and natural history of BE and IMGEJ in children. METHODS: Data from 20 BE patients (70% boys, mean age: 14.9 years) and 17 IMGEJ patients (71% boys, mean age: 14 years) were retrospectively obtained from chart review. Endoscopic and pathologic findings from index and follow-up endoscopies were analyzed. RESULTS: Most patients (70% BE and 59% IMGEJ) had underlying conditions which put them at risk for gastroesophageal reflux disease. Increased body mass index (BMI) was observed in patients without underlying conditions (BE: 30.1â±â9.8; IMGEJ: 23.9â±â6.3) compared with those with underlying conditions (BE: 19.6â±â7.8; IMGEJ: 16.4â±â2.1) (BE, Pâ=â0.02; IMGEJ, Pâ=â0.01). Incomplete intestinal metaplasia (IM) was the predominant histology seen in BE (80%) and IMGEJ patients (75%). Dysplasia and malignancy were not identified in the initial and follow-up biopsies. Concurrent gastric biopsies showed various findings (79% BE and 40% IMGEJ were normal), with 1 IMGEJ patient showing coexisting gastric IM (7%). Follow-up in 12 BE patients (mean follow-up time 51.6 months) showed 100% persistent endoscopic disease and 58% persistent IM histologically. Three of 6 IMGEJ patients (mean follow-up time 24 months) demonstrated endoscopic and histologic features consistent with BE on subsequent procedures. Moreover, a subset of BE (57%) and IMGEJ patients (67%) who underwent endoscopy before initial diagnosis showed nongoblet columnar mucosa above the anatomic gastroesophageal junction. CONCLUSIONS: Increased BMI may be a risk factor for BE and IMGEJ in pediatric patients without underlying conditions. Nongoblet columnar metaplasia and IMGEJ might represent incomplete forms of BE. Our data suggest that these patients should be closely monitored.
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Esófago de Barrett , Reflujo Gastroesofágico , Adolescente , Biopsia , Niño , Unión Esofagogástrica , Femenino , Reflujo Gastroesofágico/etiología , Humanos , Masculino , Metaplasia , Estudios RetrospectivosRESUMEN
Juvenile polyposis syndrome is a rare autosomal dominant condition characterized by multiple hamartomatous polyps throughout the gastrointestinal tract. Juvenile polyposis of infancy is a generalized severe form of juvenile polyposis syndrome associated with a poor prognosis. A 47-month-old female infant presented initially with gastrointestinal bleeding and protein-losing enteropathy at 4 months of age. At the age of 12 months, the condition worsened, requiring albumin infusions every 24 to 48 hours and red blood cell transfusions every 15 days. Upper gastrointestinal endoscopy, colonoscopy, and small-bowel enteroscopy revealed diffuse polyposis that was treated with multiple endoscopic polypectomies. Despite subtotal colectomy with ileorectal anastomosis, protein-losing enteropathy and bleeding persisted, requiring continued blood transfusions and albumin infusions. A chromosomal microarray revealed a single allele deletion in chromosome 10q23, involving both the PTEN and BMPR1A genes. Loss of PTEN function is associated with an increased activation of the protein kinase B (AKT)/mammalian target of rapamycin (mTOR) pathway involved in cell proliferation. Treatment with sirolimus, an mTOR inhibitor, was initiated with the aim of inhibiting polyp growth. Soon after initiation of treatment with sirolimus, blood and albumin infusions were no longer needed and resulted in improved patient growth and quality of life. This case represents the first detailed report of successful drug therapy for life-threatening juvenile polyposis of infancy.
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Inmunosupresores/uso terapéutico , Poliposis Intestinal/congénito , Síndromes Neoplásicos Hereditarios/diagnóstico , Síndromes Neoplásicos Hereditarios/tratamiento farmacológico , Sirolimus/uso terapéutico , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Poliposis Intestinal/diagnóstico , Poliposis Intestinal/tratamiento farmacológico , Poliposis Intestinal/cirugía , Síndromes Neoplásicos Hereditarios/cirugía , Resultado del TratamientoRESUMEN
OBJECTIVES: Variceal hemorrhage (VH) is a serious complication of portal hypertension (PH). We evaluated the feasibility, safety, and clinical impact of esophageal capsule endoscopy (ECE) in pediatric and young adult patients with known or suspected PH. METHODS: Children and young adults with PH at Boston Children's Hospital (2005-2017) were offered ECE for variceal screening or surveillance. Patient histories, ECE findings, and clinical outcomes were reviewed retrospectively. RESULTS: One hundred and forty-nine ECE studies were performed in 98 patients (57.1% male patients) using 3 ECE devices for variceal screening (66.5%) or surveillance (33.5%). Three readers interpreted the studies (88.3%, 10.3%, and 1.4%, respectively). Median age was 16 years (IQR 13.7-18.5). One hundred and three ECE studies involved patients <18 years (69.1%). Fifteen patients (29 ECE studies) had a gastrointestinal (GI) bleeding (GIB) history, 5 in the preceding 12 months.Sixty-two ECE studies (44.9%) detected varices: 59 esophageal (40 small, 19âmedium/large), 17 gastric, 6 duodenal. Other findings included: portal gastropathy (25, 18.1%), esophagitis (20, 14.5%), ulcers (5, 3.6%), erosions (31, 22.5%), heterotopic tissue (13, 9.4%), blood flecks (23, 16.7%), and mucosal scars (17, 12.3%). There were 2 transient capsule retentions and no major adverse events.ECE led to follow-up EGD in 11 (7 variceal banding) and medication initiation in 12 (4 proton-pump inhibitor, 7 nonselective beta blocker, 2 other) cases. Four patients had GIB within 12 months of ECE. CONCLUSION: ECE is a feasible alternative to EGD for screening and surveillance of esophageal varices in children and young adults.
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Endoscopía Capsular/métodos , Várices Esofágicas y Gástricas/diagnóstico por imagen , Hemorragia Gastrointestinal/diagnóstico , Hipertensión Portal/complicaciones , Adolescente , Niño , Várices Esofágicas y Gástricas/etiología , Estudios de Factibilidad , Femenino , Hemorragia Gastrointestinal/prevención & control , Humanos , Masculino , Estudios Retrospectivos , Adulto JovenRESUMEN
BACKGROUND/PURPOSE: Achalasia is an extremely rare disease in children (0.1 per 100,000 individuals). Standard treatments for this include pneumatic dilation and esophagomyotomy. Minimally invasive esophagomyotomies have increasingly been used owing to improved postoperative pain and length of stay. We describe our experience with thoracoscopic esophagomyotomy in this population. METHODS: This is a retrospective cohort study of all patients at our institution who underwent thoracoscopic esophagomyotomy for achalasia from 1995 to 2016. We used endoscopic guidance during all procedures. No fundoplication was performed. RESULTS: Thirty-one patients were included in this study. Thirteen patients underwent pneumatic dilations prior to their operation with a median of 3 dilations. Two patients had a mucosal injury during the case. There were no conversions to an open procedure. Median length of stay was 2â¯days. After the procedure, 97% of patients had initial symptom relief. Eight patients (26%) required postoperative pneumatic dilations for recurrent symptoms; there was a greater chance of this (OR 8.5) if they had a preoperative dilation. No patients required a fundoplication for reflux postoperatively. CONCLUSIONS: Thoracoscopic esophagomyotomy is a safe and effective procedure for achalasia in the pediatric population. It should be considered as an alternative to the laparoscopic approach for these patients. LEVEL OF EVIDENCE: IV.