E. Cerchia

Herlyn–Werner–Wunderlich syndrome: An “early” onset case report and review of Literature

Highlights • We describe a case of a 3 years old girl, with all features of Herlyn–Werner–Wunderlich syndrome (HWWS) who came to our attention for lower abdominal mass.• Only six cases are reported in Literature with early onset of this syndrome under 5 years.• 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.• An error of evaluation with planning of removal of mass, that can damage patients in term of chance for a successful reproductive outcome.• 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 cisto-vaginoscopy, if it is necessary. A high level of suspicion, indeed, is the key to early diagnosis.

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 childrens 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 childs hospital stay, with a view to optimizing treatment and care, preventing behavioural alterations and enhancing the childs overall life quality.

Thoracoscopic Thymectomy in Children: Our Preliminary Experience

INTRODUCTION The aim of this study is to present a preliminary series of six children affected by an anterior mediastinal mass, resulting in a thymoma. We treated this pediatric population by a video-assisted thoracoscopic thymectomy. PATIENTS AND METHODS We performed a retrospective study from January 2000 to January 2012 of all children affected by an anterior mediastinal mass, resulting in a thymoma. Data included sex, age at surgery, clinical and radiological features, surgical procedure, follow-up, and complications. RESULTS Video-assisted thoracoscopic thymectomy was performed in six children (five boys and one girl). Four patients presented with dyspnea and/or thoracic pain treated with analgesic and corticosteroid therapy without any benefits. Two patients were asymptomatic. Preoperatively, exams included radiological imaging, blood, and immunological test. All 6 patients were treated with a thoracoscopic approach; of these patients, 5 were treated with a thoracoscopic right-side thymectomy and 1 with a left-side approach. Patients were treated by a three-trocar technique, and thymectomy was performed using Valleylab (now Covidien) (Boulder, CO) LigaSure™. The mean operative time was 120 minutes. The mean hospital stay was 5 days. In all cases histological findings revealed no malignancy. The mean follow-up was 38 months. DISCUSSION Thoracoscopic thymectomy facilitated the goal of early thymectomy. The right-side approach is preferred because it gains a good visualization of the venous anatomy for dissection. The left side allows a good extended resection of both the thymus and perithymic fat. Thoracoscopic thymectomy should be consider a valid, less invasive alternative to the most radical open approach.

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.

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.

Sternal reconstruction by extracellular matrix: a rare case of phaces syndrome

Abstract Congenital defects of the sternum are rare and due to a failure of midline development and fusion of the sternal bones. Surgical correction of a sternal cleft should be preferred during infancy for functional reasons. Chest wall reconstruction represented a complex problem in the last decades. We report our successful outcome of sternal reconstruction in a rare case of PHACES syndrome, in which the patient was submitted to reconstruction of the sternum and complete closure of the thoracic defect by the employ of an extracellular matrix XCM Biologic tissue matrix. We promote the use of extracellular matrix in surgical reconstruction of chest defects for its maneuverability, plasticity, tolerability and the possibility of growing with the children’s chest getting a good compliance and optimal cosmetic results.

Pediatric Thoracoscopy and Bronchial Blockers: The Continued Search for the Ideal One-Lung Ventilation

INTRODUCTION Thoracoscopic surgical procedures in small infants and children require a growing demand for lung isolation in pediatric anesthesia practice. MATERIALS AND METHODS Between January 2006 and September 2014, 16 children underwent thoracoscopic procedures that needed one-lung ventilation using a bronchial blocker. RESULTS The thoracoscopic procedure was performed at a mean age of 99 months (range, 6-186 months) and a mean weight of 33 kg (range, 7-68 kg) without any conversion to open surgery in any case. We were able to achieve one-lung ventilation in all patients. The quality of lung deflation was excellent in all patients except in 1 due to malposition of the bronchial blocker, whereas dislodgement of this device occurred in 2 cases. CONCLUSIONS The use of a bronchial blocker provided a highly effective means of one-lung ventilation for children undergoing the thoracoscopic approach.

Circumcision with Glubran ® 2 in children: experience of Italian Center

BACKGROUND Circumcision is one of the most common surgical procedures in the world. Despite it is known its wide prevalence for religious and medical reasons in children, it remains a controversial practice in paediatric age. To date, there is no described the gold standard technique to circumcise paediatric patients. We started to use glue for circumcision about 2 years ago. We designed this prospective study with the aim to compare two surgical techniques, which were used in our hospital to perform circumcision in children. The implication for practice was the understanding if there were differences between these approaches related to patients and parents benefits to manage this condition and benefits for surgeon and hospital in term of saving money and time. METHODS This is a randomized, single-blind one-center study. It was conducted at the Department of Paediatric Surgery of Siena. Data were collected between March 2011 and December 2012. Studys population involved all patients who required circumcision. Two randomizes groups: group one which involved patients who underwent circumcision using sutures and group two, which involved patients who underwent circumcision using surgical glue (Glubran(®) 2). Two exclusion criteria were used: the redo-circumcision and the allergy or hyper-sensibility to cyanoacrylate (main component of glue). RESULTS We report 99 patients who underwent circumcision with Glubran(®) 2 in comparison with a group of children circumcised with sutures (vycril rapide). We measured three outcomes (operating time, postoperative pain and assessment of cosmetic), which, even if not all statistically significant, allowed us to draw any conclusions about the use of glue in circumcision. CONCLUSIONS Traditional circumcision is performed using a standard sleeve technique with sutures for the approximation of the skin edges. However, since some years a tissue adhesive as N-butyl-2-cyanoacrylate (NBCA) (Glubran(®) 2) is used in many centers to circumcise children. Based on our results we can conclude that, glue (Glubran(®) 2) application is an excellent alternative to circumcision in paediatric age for a faster surgery, less postoperative pain and good early cosmetic.

Split-appendix technique: Alternative urinary diversion for pediatric complete incontinence

We report our series of selected patients with complete incontinence in whom the appendix was divided and utilized for creating two continent catheterizable stomas. All patients were treated for urinary and fecal incontinence by split appendix technique. The appendix was divided into two different parts preserving adequate perfusion and used for creating an appendicocecostomy (ACE) and an appendicovesicostomy at the same time. After a clinical and radiological follow up, our patients referred a good acceptance and an easily management of the stomas in order to stay dry for all day from urine and feces with improving of their quality of life. The combination of ACE and Mitrofanoff principle have revolutionized the management of urinary and fecal incontinence in patients who are unable to utilize their urethra to keep themselves dry.

Serum levels of inhibin B in adolescents after varicocelelectomy: A long term follow up

Abstract Introduction To study the impact on adult’s fertility of serum inhibin B levels in adolescent patients with idiopathic varicocele after minimally invasive surgical correction and to compare fluctuation of pituitary-testis hormonal values and testicular volumes. Materials and Methods: A case-control study was carried out on a group adolescent patients (n=60) affected by idiopathic left varicocele (group V) and compared with control adolescents (n=40) in the Paediatric Surgery Section of Siena (from June 1993 till September 2013). Inhibin B levels and testicular volume before (T0) and after at 6 and 12 months from surgery (T1 and T2) were evaluated. Results: A positive correlation between testicular growth at T1 and T2 (P<0.001) was found. Linear regression analysis showed a positive correlation between inhibin B levels and testicular volume (expressed as the sum of the right and left values) (P<0.0001). Conclusions: Inhibin B levels are a valid marker for studying the effects of varicocele on the testicular function and confirm the necessity of early surgical correction for preventing the trophic testicular damage and male infertility.