Pediatric Reports <p>The aim of <strong>Pediatric Reports</strong> is to provide to specialists involved in clinical practice, clinical and basic research, a forum for sharing the results of their research.<br>The journal covers all aspects of pediatrics, including subspecialties but also epidemiology and social issues related to public medicine for children and adolescents.</p> PAGEPress Scientific Publications, Pavia, Italy en-US Pediatric Reports 2036-749X <p><strong>PAGEPress</strong> has chosen to apply the&nbsp;<a href="" target="_blank" rel="noopener"><strong>Creative Commons Attribution NonCommercial 4.0 International License</strong></a>&nbsp;(CC BY-NC 4.0) to all manuscripts to be published.<br><br> An Open Access Publication is one that meets the following two conditions:</p> <ol> <li>the author(s) and copyright holder(s) grant(s) to all users a free, irrevocable, worldwide, perpetual right of access to, and a license to copy, use, distribute, transmit and display the work publicly and to make and distribute derivative works, in any digital medium for any responsible purpose, subject to proper attribution of authorship, as well as the right to make small numbers of printed copies for their personal use.</li> <li>a complete version of the work and all supplemental materials, including a copy of the permission as stated above, in a suitable standard electronic format is deposited immediately upon initial publication in at least one online repository that is supported by an academic institution, scholarly society, government agency, or other well-established organization that seeks to enable open access, unrestricted distribution, interoperability, and long-term archiving.</li> </ol> <p>Authors who publish with this journal agree to the following terms:</p> <ol> <li>Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.</li> <li>Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.</li> <li>Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work.</li> </ol> Surgical approach to giant ovarian masses in adolescents: technical considerations Ovarian neoplasms arising from the surface epithelium are rare in the pediatric population; their knowledge is therefore limited and the appropriate management is poorly defined. We describe our experience and suggest our surgical approach to adolescents affected by voluminous ovarian masses. Two 15-year-old adolescents were admitted to our institution in 2017 for multilobulated, fluid-filled masses measuring over 30 cm arising from the ovaries. The cystic component was drained intraoperatively with a spillage-free technique, consisting in the application of a sterile autoadhesive transparent drape on the cyst and the insertion of a 12 Ch pleural drain, secured with a purse-string suture. Unilateral salpingo-oophorectomy was then carried out. Histology revealed mucinous cystadenoma in both patients. Surgical treatment of ovarian masses should aim at both radically excising the tumor and preserving the fertility of the patients. Decompression with spillage-free techniques can be useful to achieve radical therapy with limited manipulation of tissues. Giorgio Persano Elisa Severi Noemi Cantone Filippo Incerti Enrico Ciardini Bruno Noccioli ##submission.copyrightStatement## 2018-09-25 2018-09-25 10 3 10.4081/pr.2018.7752 Recurrent supraventricular tachycardia and necrotizing enterocolitis: A causative role or a simple association? A case report and literature review Insufficiency in mesenteric flow is a risk factor for the development of necrotizing enterocolitis (NEC). Recurrent episodes of supraventricular tachycardia (SVT) can lead to gut ischemia, with subsequent reperfusion injury. We present a term infant who developed NEC at 37 days of life after refractory SVT and reverted to sinus rhythm on day 23 and 25 of life. Resected stenotic ileum and transverse colon demonstrated inflammation with ischemia. This is the first case of NEC following SVT in a term infant without a congenital morphologic abnormality. In view of the temporal sequence of events, and in the absence of other risk factors for NEC, the SVT and NEC were likely causally related. Ghassan Nakib Sameer Sajwani Zahreddin Abusalah Abdelazeim Abdallah Nada Ibrahim Aseel Fattah Rossana Bussani Valeria Calcaterra Gloria Pelizzo ##submission.copyrightStatement## 2018-09-25 2018-09-25 10 3 10.4081/pr.2018.7636 Takayasu arteritis with an initial presentation of chronic monoarthritis mimicking oligoarticular juvenile idiopathic arthritis Patients with Takayasu arteritis (TA) generally present with non-specific symptoms that, if unrecognized and untreated, may develop vessel stenosis and/or aneurysm. There is limited data regarding chronic monoarthritis as the initial presentation in children with TA. We report a 6-yearold girl diagnosed and treated as oligoarticular juvenile idiopathic arthritis (JIA). She later developed stroke with malignant hypertension and was definitively diagnosed with TA. She additionally developed proteinuria secondary to focal segmental glomerulosclerosis. This is the report of a patient with chronic monoarthritis mimicking oligoarticular JIA which chronic monoarthritis was the presentation of TA. Since JIA is a diagnosis of exclusion, any atypical features of oligoarticular JIA should illuminate the possibility of an alternative diagnosis. Our literature review focused on musculoskeletal presentations of children with TA. Maynart Sukharomana Sorawit Viravan Nuntawan Piyaphanee Sirirat Charuvanij ##submission.copyrightStatement## 2018-07-09 2018-07-09 10 3 10.4081/pr.2018.7648 A case of atypical systemic primary carnitine deficiency in Saudi Arabia Systemic primary carnitine deficiency (SPCD) is an autosomal recessive inborn error of fatty acid metabolism caused by a defect in the transporter responsible for moving carnitine across plasma membrane. The clinical features of SPCD vary widely based on the age of onset and organs involved. During infancy, patients might show episodes of hypoketotic hypoglycemia, hepatomegaly, elevated transaminases, and hyperammonemia. Skeletal myopathy, elevated creatine kinase, and cardiomyopathy are the main manifestations in children with SPCD, while in adults, the disorder is usually manifested as cardiomyopathy, arrhythmias, or fatigability. Here, we report a 5-year-old boy with SPCD that presented as dilated cardiomyopathy with atypical features, such as anemia, respiratory distress, and proximal muscle weakness. This report supports considering carnitine deficiency treatment in the work-up of unexplained pediatric dilated cardiomyopathy. Abdulrahman Alghamdi Hani Almalki Aiman Shawli Rahaf Waggass Fahad Hakami ##submission.copyrightStatement## 2018-06-27 2018-06-27 10 3 10.4081/pr.2018.7705 Rapunzel syndrome: how to orient the diagnosis Rapunzel syndrome is a rare form of tricobezoar with a tail extending from the stomach into the small bowel; surgical removal is generally required. About 60 cases have been reported and described in the literature since 1968. We present the case of an 8-year-old girl who, during the course of a genetic consultation, was initially assigned with a clinical suspicion of ectodermal dysplasia. Surgical intervention, which resulted in the extraction of a tricobezoar of enormous size and extension, led us to the diagnosis of Rapunzel syndrome. The possibility of a tricobezoar should be considered in all cases of adolescent patients who present signs of intestinal occlusion or sub-occlusion, suffer from psychiatric disorders, and have a history of trichotillomania. Endoscopic methodology, medical imaging and clinical diagnostics are fundamental for a differential diagnosis. Psychiatric follow-up is advised to prevent recurrence. Enrico Finale Piergiorgio Franceschini Cesare Danesino Michelangelo Barbaglia Andrea Guala ##submission.copyrightStatement## 2018-06-27 2018-06-27 10 3 10.4081/pr.2018.7689