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Arrhythmic Risk in paediatric Patients Undergoing Surgical Repair for Pulmonary Atresia with Intact Ventricular Septum

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Congenital Heart Disease 2021年第1期16卷 85-94页

作者： Pietro Paolo Tamborrino Corrado Di Mambro Cecilia Marcolin Walter Vignaroli Giulia Cafiero Gianluca Brancaccio Sonia Albanese Massimo Stefano Silvetti Adriano Carotti Fabrizio Drago paediatric Cardiology and Cardiac Arrhythmia Unit Department of Paediatric Cardiology and Cardiac SurgeryBambino GesùChildren’s HospitalIRCCSRomeItaly European Reference Network for Rare and Low Prevalence Complex Disease of the Heart paediatric Cardiac surgery Unit Department of Paediatric Cardiology and Cardiac SurgeryBambino GesùChildren’s HospitalIRCCSRomeItaly Sport Medicine Unit Department of Paediatric Cardiology and Cardiac SurgeryBambino GesùChildren’s HospitalIRCCSRomeItaly

Introduction:While previous studies only focused on the arrhythmic risk associated with specific correction strategies,this study evaluates this risk in a large cohort of paediatric patients with all phenotypes of PA-IVS after surgical ***:In this single centre observational cohort study,we retrospectively evaluated 165 patients with a diagnosis of PA-IVS and we excluded those with an exclusively percutaneous treatment,patients lost or with insufficient follow-up and those affected by other arrhythmic *** history and clinical outcomes were ***:86 patients were included in the study(54 male[62.8%],mean age 16.4±6.1 years),with median follow-up from definitive repair of 12.8 years(6.4–18.9 years).They underwent three different final repairs:23 patients(26.7%)univentricular palliation,43(50%)biventricular correction,and 20(23.3%)one and a half ventricle *** patients(15%)developed arrhythmia:6 patients(all the subgroups)sinus node disfunction(SND);2(biventricular repair)premature ventricular complexes;2(one and a half ventricle repair)non-sustained ventricular tachycardia;1(biventricular repair)intra-atrial re-entrant tachycardia;1(one and a half ventricle repair)supraventricular tachyarrhythmia;1(biventricular repair)atrial *** patients with SND needed a pacemaker *** Fontan circulation showed an association with SND,while the other two groups heterogeneous types of ***:The low arrhythmic risk is related to surgical repair,it does not appear to be associated with native cardiomyopathy,and it appears to increase with length of follow *** follow-up in specialized centres is necessary to make an early diagnosis and to manage the potential haemodynamic impact at medium-long term.

关键词： Pulmonary atresia with intact ventricular septum arrhythmic risk univentricular palliation Fontan circulation biventricular repair one and a half repair

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Colonoscopy in Hong Kong Chinese children

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World Journal of Gastroenterology 2010年第9期16卷 1119-1122页

作者： Yuk Him Tam Kim Hung Lee Kin Wai Chan Jennifer Dart Yin Sihoe Sing Tak Cheung Jennifer Wai Cheung Mou Division of paediatric surgery & paediatric Urology Department of SurgeryPrince of Wales HospitalThe Chinese University of Hong Kong

AIM:To investigate the safety and diagnostic yield of colonoscopy in Chinese children in whom the procedure is not often ***:We conducted a retrospective review of all colonoscopies in consecutive children who underwent their fi rst diagnostic colonoscopy from Jan 2003 to ***:Seventy-nine children (48 boys,31 girls;mean age 9.2 ± 4.2 years) were identified and reviewed with a total of 82 colonoscopies *** caecal and ileal intubation rates were 97.6% and 75.6% *** patients (50.6%) had a positive diagnosis made in colonoscopy and that included colonic polyps (23),Crohn's disease (12),ulcerative colitis (1),and miscellaneous causes (4).80% of polyps were in the rectosigmoid *** but one were juvenile hamartomatous *** exceptionwas an adenomatous *** mean ages for children with inflammatory bowel disease (IBD) and polyps were 11.3 and 4.3 years *** was no procedure-related ***:Colonoscopy is a safe procedure in our Chinese *** increasing diagnosis of IBD in recent decades may reflect a rising incidence of the disease in our children.

关键词： Colonoscopy Children Inflammatory bowel disease Colonic polyps

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Laparoscopic approach to Meckel's diverticulum

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World Journal of Gastroenterology 2014年第25期20卷 8173-8178页

作者： Alfonso Papparella Fabiano Nino Carmine Noviello Antonio Marte Pio Parmeggiani Ascanio Martino Giovanni Cobellis Departments of paediatrics Paediatric Surgery Faculty of Medicine Paediatric Surgery-Second University of Naples80138 NaplesItaly paediatric surgery Unit-Salesi Children Hospital 60121 AnconaItaly

AIM: To retrospective review the laparoscopic management of Meckel Diverticulum(MD) in two Italian Pediatric surgery ***: Between January 2002 and December 2012, 19 trans-umbilical laparoscopic-assisted(TULA) procedures were performed for suspected MD. The children were hospitalized for gastrointestinal bleeding and/or recurrent abdominal pain. Median age at diagnosis was 5.4 years(range 6 mo-15 years). The study included 15 boys and 4 girls. All patients underwent clinical examination, routine laboratory tests, abdominal ultrasound and technetium-99m pertechnetate scan, and patients with bleeding underwent gastrointestinal endoscopy. The abdominal exploration was performed with a 10 mm operative laparoscope. Pneumoperitoneum was establishedbased on the body weight. Systematic overview of the peritoneal cavity allowed the ileum to be grasped with an atraumatic instrument. The complete exploration and surgical treatment of MD were performed extracorporeally, after intestinal exteriorization through the umbilicus. All patients' demographics, main clinical features, diagnostic investigations, operative time, histopathology reports, conversion rate, hospital stay and complications were registered and ***: MD was identified in 17 patients, while 1 had an ileal duplication and 1 a jejunal hemangioma. Fifteen patients had painless intestinal bleeding, while 4 had recurrent abdominal pain and exhibited cyst like structures in an ultrasound study. Eleven patients had a positive technetium-99m pertechnetate scan. In the patients with bleeding, gastrointestinal endoscopy did not name the source of hemorrhage. All patients were subjected to a TULA surgical procedure. An intestinal resection/anastomosis was performed in 14 patients, while 4 had a wedge resection of the diverticulum and 1 underwent stapling diverticulectomy. All surgical procedures were performed without conversion to open laparotomy. Mean operative time was 75 min(range 40-115 min). No m

关键词： Meckel's diverticulum Laparoscopy One trocar surgery Gastrointestinal bleeding Minimal invasive surgery Ileal duplication Jejunal hemangioma

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Laparoscopic excision of Meckel's diverticulum in children:What is the current evidence?

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World Journal of Gastroenterology 2014年第41期20卷 15158-15163页

作者： Kin Wai Edwin Chan Kim Hung Lee Hei Yi Vicky Wong Siu Yan Bess Tsui Yuen Shan Wong Kit Yi Kristine Pang Jennifer Wai Cheung Mou Yuk Him Tam Division of paediatric surgery and paediatric Urology Department of SurgeryThe Prince of Wales HospitalThe Chinese University of Hong Kong

Complications aroused from Meckel’s diverticulum tend to developed in children. Children presented with abdominal pain, intestinal obstruction, intussusception or gastrointestinal bleeding may actually suffered from complicated Meckel’s diverticulum. With the advancement of minimally invasive surgery (MIS) in children, the use of laparoscopy in the diagnosis and subsequent laparoscopic excision of Meckel’s diverticulum has gained popularity. Recently, single incision laparoscopic surgery (SILS) has emerged as a new technique in minimally invasive surgery. This review offers the overview in the development of MIS in the management of children suffered from Meckel’s diverticulum. The current evidence in different laparoscopic techniques, including conventional laparoscopy, SILS, the use of special laparoscopic instruments, intracorporeal diverticulectomy and extracorporeal diverticulectomy in the management of Meckel’s diverticulum in children were revealed.

关键词： Laparoscopy Meckel’ s diverticulum Children

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Surgical strategies in paediatric inflammatory bowel disease

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World Journal of Gastroenterology 2015年第20期21卷 6101-6116页

作者： Colin T Baillie Jennifer A Smith Department of paediatric surgery Royal Liverpool Childrens Hospital

Inflammatory bowel disease(IBD) comprises two distinct but related chronic relapsing inflammatory conditions affecting different parts of the gastrointestinal tract. Crohn's disease is characterised by a patchy transmural inflammation affecting both small and large bowel segments with several distinct phenotypic presentations. Ulcerative colitis classically presents as mucosal inflammation of the rectosigmoid(distal colitis), variably extending in a contiguous manner more proximally through the colon but not beyond the caecum(pancolitis). This article highlights aspects of the presentation, diagnosis, and management of IBD that have relevance for paediatric practice with particular emphasis on surgical considerations. Since 25% of IBD cases present in childhood or teenage years, the unique considerations and challenges of paediatric management should be widely appreciated. Conversely, we argue that the organizational separation of the paediatric and adult healthcare worlds has often resulted in late adoption of new approaches particularly in paediatric surgical practice.

关键词： Inflammatory bowel disease Ulcerativecolitis Crohn's disease paediatric surgery

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Abdominal cocoon in children: A case report and review of literature

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World Journal of Gastroenterology 2021年第37期27卷 6332-6344页

作者： Daniel Keese Andrea Schmedding Kerstin Saalabian Georgy Lakshin Henning Fiegel Udo Rolle Goethe-University Frankfurt Department of Paediatric Surgery and Paediatric UrologyUniversity HospitalFrankfurt 60590Germany

BACKGROUND Abdominal cocoon or“encapsulating peritoneal sclerosis”(EPS)is an uncommon and rare cause of intestinal *** a few cases have been reported in paediatric ***,EPS is described as the primary form in young adolescent girls from tropical and subtropical countries because of viral peritonitis due to retrograde menstruation or a history of peritoneal *** patients are asymptomatic or present with abdominal pain,which is likely to occur secondary to subacute bowel *** at imaging,such as ultrasound,computed tomography,and magnetic resonance imaging,are often *** diagnosed,EPS is characterized by total or partial encasement of the bowel within a thick fibrocollagenous membrane that envelopes the small intestine in the form of a cocoon because of chronic intraabdominal fibroinflammatory *** membrane forms a fibrous tissue sheet that covers,fixes,and finely constricts the gut,compromising its *** SUMMARY We present a case of EPS in a 12-year-old boy 8 wk after primary surgery for resection of symptomatic jejunal *** was no history of peritoneal dialysis or drug *** In this report,we sought to highlight the diagnostic,surgical,and histopathological characteristics and review the current literature on EPS in paediatric patients.

关键词： Abdominal cocoon Peritoneal encapsulation Encapsulating peritoneal sclerosis Intestinal obstruction Children Case report

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Use of ketamine sedation for the management of displaced paediatric forearm fractures

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World Journal of Orthopedics 2018年第3期9卷 50-57页

作者： Anatole Vilhelm Wiik Poonam Patel Joanna Bovis Adele Cowper Philip Socrates Pastides Alison Hulme Stuart Evans Charles Stewart Department of surgery and Cancer Charing Cross Hospital Department of paediatric Emergency Chelsea and Westminster Hospital Department of Trauma and Orthopaedics Chelsea Westminster Hospital

AIM To determine if ketamine sedation is a safe and cost effective way of treating displaced paediatric radial and ulna fractures in the emergency department. METHODS Following an agreed interdepartmental protocol, fractures of the radius and ulna(moderately to severely displaced) in children between the age of 2 and 16 years old, presenting within a specified 4 mo period, were manipulated in our paediatric emergency department. Verbal and written consent was obtained prior to procedural sedation to ensure parents were informed and satisfied to have ketamine. A single attempt at manipulation was performed. Pre and postmanipulation radiographs were requested and assessed to ensure adequacy of reduction. Parental satisfaction surveys were collected after the procedure to assess the perceived quality of treatment. After closed reduction and cast immobilisation, patients were then followed-up in the paediatric outpatient fracture clinic and functional outcomes measured prospectively. A cost analysis compared to more formal manipulation under a general anaesthetic was also *** During the 4 mo period of study, 10 closed, moderate to severely displaced fractures were identified and treated in the paediatric emergency department using our ketamine sedation protocol. These included fractures of the growth plate(3), fractures of both radius and ulna(6) and a single isolated proximal radius fracture. The mean time from administration of ketamine until completion of the moulded plaster was 20 min. The mean time interval from sedation to full recovery was 74 min. We had no cases of unacceptable fracture reduction and no patients required any further manipulation, either in fracture clinic or under a more formal general anaesthetic. There were no serious adverse events in relation to the use of ketamine. Parents, patients and clinicians reported extremely favourable outcomes using this technique. Furthermore, compared to using a manipulation under general anaesthes

关键词： paediatrics Forearm fractures Displaced fractures Ketamine Salter Harris Procedural sedation

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Upper gastrointestinal tract involvement of pediatric inflammatory bowel disease: A pathological review

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World Journal of Gastroenterology 2019年第16期25卷 1928-1935页

作者： Dua Abuquteish Juan Putra Division of Pathology Department of Paediatric Laboratory Medicine Hospital for Sick Children

Upper gastrointestinal(UGI) tract involvement of inflammatory bowel disease(IBD) is commonly seen in pediatric patients. Upper endoscopy is included in the routine workup of children with suspected IBD to enhance the diagnosis and management of these patients. Currently, childhood IBD is classified into ulcerative colitis(UC), atypical UC, Crohn's disease(CD) and IBD *** confirmation of UGI tract involvement, in particular the presence of epithelioid(non-caseating) granulomas, is helpful in confirming the diagnosis of IBD and its classification. Herein, we reviewed selected IBD-associated UGI tract manifestations in children. Lymphocytic esophagitis, seen predominantly in CD,is histologically characterized by increased intraepithelial lymphocytes(> 20 in one high-power field) in a background of mucosal injury with absence of granulocytes. Focally enhanced gastritis is a form of gastric inflammation in pediatric IBD marked by a focal lymphohistiocytic pit inflammation with or without granulocytes and plasma cells in a relatively normal background gastric mucosa. Duodenal inflammation seen in children with IBD includes cryptitis,villous flattening, increased intraepithelial lymphocytes, and lamina propria eosinophilia. Finally, epithelioid granulomas not associated with ruptured gland/crypt are a diagnostic feature of CD. The clinicopathologic correlation and differential diagnosis of each microscopic finding are discussed. Clinicians and pathologists should be cognizant of the utility and limitations of these histologic features.

关键词： Pediatric Inflammatory bowel disease Lymphocytic esophagitis Focally enhanced gastritis Epithelioid granuloma Crohn’s disease Ulcerative colitis

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Differential changes in intrinsic innervation and interstitial cells of Cajal in small bowel atresia in newborns

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World Journal of Gastroenterology 2010年第45期16卷 5716-5721页

作者： Stefan Gfroerer Roman Metzger Henning Fiegel Priya Ramachandran Udo Rolle Department of paediatric surgery Johann Wolfgang Goethe-University Frankfurt/MTheodor-Stern-Kai 760590 Frankfurt/MGermany Department of paediatric surgery University of Leipzig04103 LeipzigGermany Department of Pediatric surgery Kanchi Kamakoti Childs Trust Hospital12-A ChennaiIndia

AIM: To investigate morphological changes of the enteric nervous system (ENS) and the interstitial cells of Cajal (ICCs) in small bowel ***: Resected small bowel specimens from affected patients (n = 7) were divided into three parts (proximal, atretic, distal). Standard histology and enzyme immunohistochemistry anti-S100, anti-protein gene product (PGP) 9.5, anti-neurofilament (NF), antic-kit-receptor (CD117) was carried out on conventional paraffin sections of the proximal and distal part. RESULTS: The neuronal and glial markers (PGP 9.5, NF, S-100) were expressed in hypertrophied ganglia and nerve fibres within the myenteric and submucosal plexuses. Furthermore, the submucous plexus contained typical giant ganglia. The innervation pattern of the proximal bowel resembled intestinal neuronal dysplasia. The density of myenteric ICCs was clearly reduced in the proximal bowel, whereas a moderate number of muscular ICCs were found. The anti-CD117 immunore- action revealed additional numerous mast cells. The distal bowel demonstrated normal morphology and density of the ENS, the ICCs and the mast ***: The proximal and distal bowel in small bowel atresia revealed clear changes in morphology and density of the ENS and ICCs.

关键词： Small bowel atresia Enteric nervous system Gastrointestinal motility Interstitial cells of Cajal

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Hepatocellular adenoma in the paediatric population:Molecular classification and clinical associations

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World Journal of Gastroenterology 2020年第19期26卷 2294-2304页

作者： Elan Hahn Juan Putra Division of Pathology Department of Paediatric Laboratory Medicinethe Hospital for Sick ChildrenToronto M5G 1X8ONCanada Department of laboratory Medicine and Pathobiology University of TorontoToronto M5S 1A8ONCanada

Hepatocellular adenomas(HCAs)represent rare,benign liver tumours occurring predominantly in females taking oral *** children,HCAs comprise less than 5%of hepatic tumours and demonstrate association with various *** contemporary classification of HCAs,based on their distinctive genotypes and clinical phenotypes,includes hepatocyte nuclear factor 1 homeobox alpha-inactivated HCAs,beta-catenin-mutated HCAs,inflammatory HCAs,combined beta-catenin-mutated and inflammatory HCAs,sonic hedgehogactivated HCAs,and unclassified *** children,there is a lack of literature on the characteristics and distribution of HCA *** this review,we summarized different HCA subtypes and the clinicopathologic spectrum of HCAs in the paediatric population.

关键词： paediatric Hepatocellular adenoma Malignant transformation Beta-catenin HNF1A Glycogen storage disorders

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