Herlyn-Werner-Wunderlich syndrome: An "early" onset case report and review of Literature.

Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital mullerian anomaly consisting of uterus didelphys, hemivaginal septum, and unilateral renal agenesis [1,2]. Most authors reported cases of Herlyn-Werner-Wunderlich syndrome with prepuberal or postpuberal onset with cyclical abdominal pain and a vaginal mass (3-8). Only six cases are reported in Literature with early onset of this syndrome under 5 years (9-14). Our case is about 3 years old girl, with all the features of this syndrome who came to our attention for lower abdominal mass. The aim of this article is to share our experience and focus the attention on the importance of high level of suspicion of HWWS in neonatal period to early diagnosis and treatment. The possible early presentation of this syndrome should be suspected in all neonates (females) with renal agenesia confirmed postnatally or with prenatal diagnosis. It is common, in fact, an error of evaluation with planning of removal of mass, that can damage patients in term of chance for a successful reproductive outcome. For all these reasons, our team consider HWWS as differential diagnosis in newborn with prenatal ultrasonography of a cystic mass behind the urinary bladder in the absence of a kidney and plan a pelvic ultrasound (with aim to identify an uterus, normal or dydhelfus, and presence or absence of pelvic mass), an examination under anesthesia and cystoscopy and vaginoscopy, if it is necessary. A high level of suspicion, indeed, is the key to early diagnosis.

Esophagogastric dissociation reduces the re-operation rate for persistent gastroesophageal reflux in severely neurologically impaired children.

PURPOSE: In this study we want to demonstrate the effectiveness of the esophagogastric dissociation as a first level operation in treatment of the gastroesophageal reflux in severe neurologically impaired children, in term of a reduction of reoperation rate. METHODS: We divided patients operated from 1998 to 2005 in a group A, composed by children treated with fundoplication, and in a group AR, composed by the patients of group A who had a recurrence of reflux and that was treated with esophagogastric dissociation. Patients operated from 2005 to 2013 were selected on the basis of the severity of the neurological impairment and were divided in a group B, treated with fundoplication, and in a group C of more severe impaired children, treated with esophagogastric dissociation. Data regarding the complications of the A and C groups were analyzed with Fisher's test. RESULTS: We evaluated 63 patients: 34 (54%) in group A, 11 in group AR, 15 (23.6%) in group B, 14 (22.4%) in group C. The Fisher's test showed a non significant difference with a p value of 0.2. CONCLUSION: Despite of statistic result we believe that TOGD is a useful procedure as the first choice of surgical management in severe neurological impaired children affected by gastroesophageal reflux.

Uncommon surgical emergencies in neonatology.

Objective. Over the past decade, multiple factors have changed the pattern of neonatal surgical emergencies. An increase in prenatal screenings and the development of neonatal tertiary care centres have changed the clinical approach to these kids. Materials and methods. Between 1995 to 2011 were retrospectively reviewed 34 patients with diagnosis of uncommon rare neonatal surgical emergencies at our institute. We analyzed: sex, gestational age, weight at birth, primary pathology, prenatal diagnosis, associated anomalies, age and weight at surgery, clinical presentation, start of oral feeding and hospitalization. The follow-up was performed at 6,12, 24 and 36 months. Results. There were 21 male and 13 female. The gestational age ranged between 28 and 36 weeks. The weight at birth ranged between 700 and 1400 grams. Oral feeding was started between 4th and 10th postoperative day. The average hospitalization was about 70.47 days. To date, all patients have finished the followup. They are healthy. Conclusion. The outcome of the patients with uncommon surgical emergencies is different based on the etiology. Overall survival is generally good but is influenced by the associated anomalies.

Preoperative distraction in children: hand-held videogames vs clown therapy.

Anxiety in children undergoing surgery is characterized by feelings of tension, apprehension, nervousness and fear which may manifest differently. Postoperative behavioural changes such as nocturnal enuresis, feeding disorders, apathy, and sleep disturbances may stem from postoperative anxiety. Some Authors pointed out that over 60% of children undergoing surgery are prone to developing behavioural alterations 2 weeks after surgery. Variables such as age, temperament and anxiety both in children and parents are considered predictors of such changes.1 Studies were published describing how psycho-behavioural interventions based on play, learning and entertainment in preparing children for surgery, may reduce preoperative anxiety. Clown-therapy is applied in the most important paediatric facilities and has proved to diminish children's emotional distress and sufferance, as well as consumption of both analgesics and sedatives and to facilitate the achievement of therapeutic goals. The aim of our study was to evaluate the efficacy of clown-therapy during the child's hospital stay, with a view to optimizing treatment and care, preventing behavioural alterations and enhancing the child's overall life quality.

Cervical Lymphadenitis by Mycobacterium triplex in an Immunocompetent Child: Case Report and Review.

Mycobacterium triplex was first described in 1996. This nontuberculous Mycobacterium causes a severe pulmonary disease in immunocompromised patients but it can involve also healthy patients. A literature search was made on the PubMed database and it produced only few cases of children with cervical lymphadenitis due to this Mycobacterium Triplex. We are describing a case of M. triplex cervical lymphadenitis in an immunocompetent child.