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A female neonate was born with asymmetric lower limbs, the right leg appearing enlarged, with thickened, reddish-purple skin and ectasic superficial reticulum (figure 1A,B). Limb pulses were present and symmetrical. The girl’s family history and prenatal scans were unremarkable. Laboratory findings were within the normal range, except for a mild thrombocytopenia (90 000/μL), which spontaneously resolved during the next few days. A leg X-ray and the Doppler analysis ruled out the presence of calcifications and venous varices, respectively. Ultrasound showed significant skin thickening, with marked dermal hypertrophy and hyperechogenicity. Magnetic resonance showed circumferential thickening of the derma, with mild hypertrophy of some perforating vessels (figure 2). A biopsy of the right thigh showed capillary malformations on histology.Figure 1(A, B) Hypertrophy of the right lower limb, with large capillary malformation extending to the gluteus and the external genitalia.Figure 2Axial THRIVE magnetic resonance scan of the thighs’ proximal third, showing circumferential dermal thickening and inhomogeneity of the right leg’s subcutaneous tissue.QuestionsBased on the clinical picture and investigations results, which is the most likely diagnosis?Beckwith-WiedemannCLOVES syndromeKlippel-Trenaunay syndromeKaposiform hemangioendotheliomaHow can the diagnosis be confirmed?CT with PETLymphoscintigraphyGenetic testingNone of the above, the diagnosis is clinicalWhat is the mainstay of management?Conservative with follow-upPharmacotherapySclerotherapySurgeryWhich of the following complications can occur?ScoliosisGlaucomaUrinary and gastrointestinal bleedingAll of the aboveAnswers can be found on page 02.

AIM In 2009, the Italian society for paediatric nephrology suggested the need for cystography, following a first febrile urinary tract infection (UTI), only in children at high risk for dilating vesicoureteral reflux or in the event of a second infection. The aim of this study was to evaluate the adequacy of the risk factors proposed by the Italian guidelines. METHODS Children aged 2-36 months, managed by 10 Italian hospitals between 2009 and 2013, with a first febrile UTI were retrospectively evaluated. RESULTS Four hundred and fourteen children were included: 51% female, mean age eight months. Escherichia coli was responsible of 84% UTIs. 269 children (65%) presented at least one risk factor, thus were further investigated: 44% had a reflux. The presence of a pathogen other than E. coli significantly predicted high-grade reflux, both in the univariate (Odd Ratio 2.52, 95% Confidence Interval 1.32-4.81, p

Objective This study aimed to assess the spectrum of pathologies responsible for torticollis in children presenting to the emergency department and to evaluate the associated symptoms to determine clinical red flags for hospitalization. Methods This was a historical retrospective cohort study. Medical records of children evaluated in our emergency department for torticollis from 2008 to 2013 were reviewed. Results Among 392 identified patients, 61% had postural torticollis,19.4% infection related, 16.3% traumatic, and 3.5% other. Twenty-five patients (6.4%) were hospitalized. Four variables were strongly and independently related to the severe outcome: fever, sore throat, headache, and age. Conclusions The association of 2 or 3 of these 4 features carried a risk of 32% and 58%, respectively, of having a severe illness.

Aim: Ambulatory surgery is a daily requirement in poor countries, and limited means and insufficient trained staff lead to the lack of attention to the patient’s pain. Midazolam is a rapid-onset, short-acting benzodiazepine which is used safely to reduce pain in children. We evaluated the practicability of intranasal midazolam sedation in a suburban hospital in Luanda (Angola), during the surgical procedures. Methods: Intranasal midazolam solution was administered at a dose of 0.5 mg/kg. Using the Ramsay’s reactivity score, we gave a score to four different types of children’s behaviour: moaning, shouting, crying and struggling, and the surgeon evaluated the ease of completing the surgical procedure using scores from 0 (very easy) to 3 (managing with difficulty). Results: Eighty children (median age, 3 years) were recruited, and 140 surgical procedures were performed. Fifty-two children were treated with midazolam during 85 procedures, and 28 children were not treated during 55 procedures. We found a significant difference between the two groups on the shouting, crying and struggling parameters (p

Hughes-Stovin Syndrome (HSS) is a very rare clinical disorder characterized by pulmonary artery aneurysms and peripheral venous thrombosis. Typical symptoms are recurrent fever, chills, haemoptysis and it usually affects young men. The natural course of the illness is usually fatal because of fulminant haemoptysis. The aetiology of Hughes-Stovin syndrome is still unknown; however it is supposed to be a clinical variant manifestation of Behcet disease.

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