Journal of Paediatric Care Insight A Deep Esophageal Injury from a Lithium Battery: A Case Report

Abstract Context: Over the last ten years the ingestion of disk batteries and its serious consequences have been increasing. The severity of injury is related to a growing diffusion of the new lithium battery that may cause catastrophic damages when lodged in the esophagus in children. Case report: A five-year-old boypresented to the Emergency Department of our tertiary pediatric Institute for a lithium battery lodged in the mid esophagus. Emergent esophagoscopy revealed a severe deep, mild bleeding ulceration of the wall in which the battery was partially wedged. The investigation was stopped and on-call cardio-vascular surgeon started left thoracotomy to exclude damages of the main vessels. With the thorax open, the endoscopy was repeated and a directional relationship between the battery and the aorta was excluded by means of transillumination. The cell, a CR2032 lithium battery, was then removed. Central line parenteral nutrition, i.v. omeprazole plus antibiotics were started with a drainage tube left in the chest. During the follow-up the child undergone several chest X-rays with the suspicion of esophageal perforation. Angio-TC done on day 7 showed air into the thickened esophageal wall and in the mediastinum with severe peri-aortic edema without lesion of the vessel. MRi performed on day 21 showed only a persistent thickening of the esophageal wall. On day 28 an esophagogram was normal and the child was discharged asymptomatic. Two months later the investigation was repeated resulting entirely normal. Conclusions: Treatment of disk battery ingestion requires a multidisciplinary approach that can be implemented only in a tertiary pediatric hospital. Surgery can play an important role. Severe complications can occur several days after battery removal

The diagnostic challenge of very early-onset enterocolitis in an infant with XIAP deficiency

Background: Aggressive course and resistance to treatments usually characterize very early onset inflammatory bowel disease (VEO-IBD). Some VEO-IBD cases are due to monogenic immune defects and can benefit from hematopoietic stem cell transplantation (HSCT). Case presentation: We describe a Caucasian male baby who presented in the first months of life macrophage activation syndrome, followed by intractable colitis, recurrent episodes of fever and mild splenomegaly. After several immunological, genetic and clinical investigations, subsequently a therapeutic attempt with colectomy, analysis of VEO-IBD-associated genes, revealed a causative mutation in XIAP. The genetic diagnosis of a primary immune deficiency allowed curing the boy with hematopoietic stem cell transplantation. Conclusion: Our report, together with novel findings from recent literature, should contribute to increase awareness of monogenic immune defects as a cause of VEO-IBD. Comprehensive genetic analysis can allow a prompt diagnosis, resulting in the choice of effective treatments and sparing useless and damaging procedures

Usefulness and safety of double endoscopy in children with gastroesophageal reflux and respiratory symptoms

SummaryBackgroundManagement of children with gastroesophageal reflux disease (GORD) and difficult-to-treat (D-T-T) respiratory symptoms may include double fiberoptic, airway and oesophago-gastro-duodenoscopies (DE). A study was performed to evaluate the usefulness and safety of DE in children with severe GORD and D-T-T respiratory symptoms.MethodsA 3-year retrospective review of records of children who underwent DE under general anaesthesia was performed: the relevant clinical information obtained and the occurrence of complications in the 72h following the DE.ResultsInflammatory changes of the airways were found at bronchoscopy in 40 out of the 60 children: bronchoalveolar lavage (BAL) demonstrated positive lipid-laden alveolar macrophages (LLAM), neutrophilic inflammation or both, respectively in 9, 12 and 16 patients. BAL bacterial cultures were positive in 2 patients with elevated airway neutrophilia. Structural airway abnormalities, explaining not GOR-related D-T-T respiratory symptoms were identified in 11 patients. Oesophagoscopic findings supporting GORD were detected in 32/60 children and confirmed by consistent histological changes in oesophageal mucosal biopsies (OEB) in 27.The frequency of complications, all minor, was low during the procedure and in the following 72h. They included mild desaturation, stridor or bronchospasm, vomiting, dysphagia and hyperthermia requiring antibiotic treatment in 1 patient. No “new onset” complication was observed after 48h following DE. The time-dependent hazard of complications was significantly higher for patients with a history of onset of respiratory symptoms early in life (≤2 years of age) (p=0.038).ConclusionDE can be useful in the clinical evaluation of children with D-T-T respiratory symptoms and GORD and is associated with low frequency of mild complications when performed by appropriately trained and experienced personnel

Multicentric Case-Control Study on Azathioprine Dose and Pharmacokinetics in Early-onset Pediatric Inflammatory Bowel Disease

BACKGROUND: Early-onset inflammatory bowel disease (IBD) is generally aggressive, with a high probability of complications and need of surgery. Despite the introduction of highly effective biological drugs, treatment with azathioprine continues to be important even for early-onset IBD; however, in these patients azathioprine response seems to be reduced. This study evaluated azathioprine doses, metabolite concentrations, and their associations with patients' age in children with IBD treated at 6 tertiary pediatric referral centers. METHODS: Azathioprine doses, metabolites, and clinical effects were assessed after at least 3 months of therapy in 17 early-onset (age 12 and <18 yrs, controls) patients with IBD. Azathioprine dose was titrated on therapeutic efficacy (response and adverse effects). Azathioprine metabolites and thiopurine methyltransferase activity were determined by high-performance liquid chromatography with ultra violet-vis detection (HPLC-UV) methods. RESULTS: Frequency of patients in remission was similar among early-onset and control groups, respectively (82% and 84%, P value = 0.72). Early-onset patients required higher doses of azathioprine (median 2.7 versus 2.0 mg\ub7kg\ub7d, P value = 1.1 7 10). Different doses resulted in comparable azathioprine active thioguanine nucleotide metabolite concentrations (median 263 versus 366 pmol/8 7 10 erythrocytes, P value = 0.41) and methylmercaptopurine nucleotide concentrations (median 1455 versus 1532 pmol/8 7 10 erythrocytes, P value = 0.60). Lower ratios between thioguanine nucleotide metabolites and azathioprine doses were found in early-onset patients (median 98 versus 184 pmol/8 7 10 erythrocytes\ub7mg\ub7kg\ub7d, P value = 0.017). Interestingly, early-onset patients presented also higher thiopurine methyltransferase activity (median 476 versus 350 nmol methylmercaptopurine/mg hemoglobin/h, P-value = 0.046). CONCLUSIONS: This study demonstrated that patients with early-onset IBD present increased inactivating azathioprine metabolism, likely because of elevated activity of the enzyme thiopurine methyltransferase

Sudden blindness in a child with Crohn’s disease

Inflammatory bowel disease (IBD) is often associated with extraintestinal manifestations (EIMs) such as optic neuritis (ON), although this has been described in only a few adult patients so far, all of whom were affected with Crohn’s disease (CD). Furthermore, ON and demyelinating diseases have been demonstrated to be more frequent in IBD patients than in control populations. In our current case report, we describe a child with active CD who developed sudden blindness due to bilateral ON that was not related to any known cause, and that promptly responded to a high dose of steroids. Investigations and a clinical follow-up have so far ruled out the development of demyelinating diseases in this patient. To our knowledge, this is the first report of ON in a pediatric patient with CD. Possible explanations for this case include an episodic EIM of an active bowel disease, an associated autoimmune disorder such as a recurrent isolated ON, the first manifestation of multiple sclerosis, or another demyelinating disease that could appear in a later follow-up

Scanning electron microscopy study of small bowel biopsies in chronic diarrhoea in chlldhood

In thi, study wc have compared the results ot Scanning Electron Microscopy (SEM) with Light- and Stereomicroscopy in a series of small bowel biop\ies in children. In 9 cases displaying features of partial or wbtotal atrophy, Light and Dissecting-Microscopy yiclded similar results. The distinction between coeliac and noncoeliac chronic diari-hoen was only possible on cliiiical grounds, and by the iininunological detection of specific antibodies. On SEM however coeliac patients showed characteristic alterations consisting of: absence of villi: prominent crypt outlets resulting in a mosaic appearance: concentric furrows running al1 around the openings; and downy brush feature at higti power. The microvilli were loosely distributed and had an irregular pleomorphic outline; they often displayed a druinstick swelling of tlic tip and were bent. In contra5t. non-coeliac chronic diarrhoea cases were characterized bv a thick mucous layer on the mucosa1 surface, that made it impossible to visualize further chan~es. U Peculiar \iascular changes in Iymphangiectasia and in sickle beta thalasseinia could be detected only by Liglit Microscopy. In addition, in the lymphangiectasia ca\e SEM allowed the detection of enteroadherent bacteria: and in the lambliasi, case, of pseudomembi-rine\. Absence of glycocalyx was noted both in controls and in patients. The results of this study point to a diagnostic utility of SEM particularly in the differential diagnosis of chronic diarrhoea; moreover they suggest tliat enteroadherent bacteria may not be pathogenic and that the absence of glycocalyx is not specific for allergic enteropathy as previously claimed

Cone-like resection, fistulectomy and mucosal rectal sleeve partial endorectal pull-through in paediatric Crohn's disease with perianal complex fistula

Background: Perianal abscesses and fistulae have been reported in approximately 15% of patients with paediatric Crohn's disease and they are associated with poor quality of life. Several surgical techniques were proposed for the treatment of perianal Crohn's disease, characterized by an elevated incidence of failure, incontinence, and relapse.Aim of our study was to present the technical details and results of our surgical technique in case of recurrent, persistent, complex perianal ano-rectal destroying Crohn's disease not responding to medical treatment. Methods: Data of patients who underwent surgical treatment (cone-like resection, fistulectomy, sphincter reconstruction, endorectal advancement sleeve flaps like in Soave endorectal pull-through) for complicated high-level trans, inter or suprasphincteric fistulae between January 2009 and June 2014 were retrospectively reviewed. Results: 20 surgical procedures were performed in 11 patients (males 72.7%) with transsphincteric (n= 5), intersphincteric (n= 4) and suprasphincteric (n= 2) fistulae. Three patients needed a second treatment. Two patients needed more than 2 surgeries and one temporary colostomy. No patient presented anal incontinence at 15 months' median follow-up. Conclusions: Although several procedures may be required to obtain a complete remission of perianal lesions, in our series the proposed surgical technique seemed effective and safe, preserving anal continence in all treated cases and reducing the need of faecal diversion

Minimally invasive surgery for paediatric inflammatory bowel disease: Personal experience and literature review

reserved9noThe incidence of paediatric inflammatory bowel disease (PIBD) has dramatically increased in the last 20 years. Although first reported in mid 1970s', diagnostic laparoscopy has started to be routinely adopted in paediatric surgical practice since late 1990s'. Minimally invasive surgery was first limited to diagnostic purposes. After 2002 it was also applied to the radical treatment of PIBD, either Crohn's disease (CD) or Ulcerative colitis. During the last decade minimally invasive approaches to PIBD have gained popularity and have recently became the "gold standard" for the treatment of such invalidating and troublesome chronic diseases. The authors describe and track the historical evolution of minimally invasive surgery for PIBD and address all available opportunities, including most recent advancements such as robotic surgery, single port approaches and minimally invasive treatment of perianal fistulising CD. A systematic review of all series of PIBD treated with minimally invasive approaches published so far is provided in order to determine the incidence and type of patients' complications reported up to present days. The authors also describe their experience with minimally invasive surgery for PIBD and will report the results of 104 laparoscopic procedures performed in a series of 61 patients between January 2006 and December 2014.mixedPini-Prato, Alessio; Faticato, Maria Grazia; Barabino, Arrigo; Arrigo, Serena; Gandullia, Paolo; Mazzola, Cinzia; Disma, Nicola; Montobbio, Giovanni; Mattioli, GirolamoPINI PRATO, Alessio; Faticato, MARIA GRAZIA; Barabino, Arrigo; Arrigo, Serena; Gandullia, Paolo; Mazzola, Cinzia; Disma, NICOLA MASSIMO; Montobbio, Giovanni; Mattioli, Girolam