Pediatric Reports 2019-01-19T09:29:54+01:00 Emanuela Fusinato Open Journal Systems <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> Surgical approach to giant ovarian masses in adolescents: technical considerations 2019-01-19T09:29:52+01:00 Giorgio Persano Elisa Severi Noemi Cantone Filippo Incerti Enrico Ciardini Bruno Noccioli 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. 2018-09-25T12:09:09+02:00 ##submission.copyrightStatement## Recurrent supraventricular tachycardia and necrotizing enterocolitis: A causative role or a simple association? A case report and literature review 2019-01-19T09:29:54+01:00 Ghassan Nakib Sameer Sajwani Zahreddin Abusalah Abdelazeim Abdallah Nada Ibrahim Aseel Fattah Rossana Bussani Valeria Calcaterra Gloria Pelizzo 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. 2018-09-25T11:56:50+02:00 ##submission.copyrightStatement## Takayasu arteritis with an initial presentation of chronic monoarthritis mimicking oligoarticular juvenile idiopathic arthritis 2018-07-18T15:23:57+02:00 Maynart Sukharomana Sorawit Viravan Nuntawan Piyaphanee Sirirat Charuvanij 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. 2018-07-09T11:33:40+02:00 ##submission.copyrightStatement## A case of atypical systemic primary carnitine deficiency in Saudi Arabia 2018-07-18T15:22:44+02:00 Abdulrahman Alghamdi Hani Almalki Aiman Shawli Rahaf Waggass Fahad Hakami 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. 2018-06-27T17:26:31+02:00 ##submission.copyrightStatement## Rapunzel syndrome: how to orient the diagnosis 2018-07-18T15:22:03+02:00 Enrico Finale Piergiorgio Franceschini Cesare Danesino Michelangelo Barbaglia Andrea Guala 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. 2018-06-27T14:00:16+02:00 ##submission.copyrightStatement##