HYPERPLASIA OF THYMIC GLAND: LEFT VIDEO-ASSISTED THORACOSCOPIC APPROACH

Hyperplasia of thymic gland is a rare benign entity that should be considered in the differential diagnosis of anterior mediastinal masses in children and young adolescents. We report a case of a patient with a thymic mass, diagnosed occasionally for respiratory symptoms and treated by video-assisted thoracoscopic surgery. A previously healthy 10 years-old boy presented to our hospital for retrosternal pain and dyspnea with restriction to daily activities from four months. Diagnostic imaging was performed, including a chest x-ray and a magnetic resonance imaging, showing a large homogeneous anterosuperior mediastinal mass, more extended on the left side. The additional laboratory analysis, considered essential for differential diagnosis with myasthenia gravis and lymphoma, resulted negative. In view of these findings, our patient underwent to video assisted thoracoscopy with left-sided approach for a total resection of thymus and perithymic fat. The patient made an excellent recovery without postoperative complications and was discharged from the hospital four days later. Histopathological examination showed a normal thymic architecture like a true thymic hyperplasia. At follow up, chest x-ray was normal in absence of pleural and parenchimal alterations. Thoracoscopic thymectomy is a safe technique that allows to achieve the goal of early thymectomy with the advantages of less invasive procedure

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

AbstractHerlyn–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

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

Caudal 'duplication' or 'split' syndrome: Is there a misnomer?

Abstract'Caudal duplication syndrome' was coined to describe the apparent duplication of organs derived from the hindgut, the neural tube and the adjacent mesoderm. Review of the anatomy suggests that the word 'duplication' may be a misnomer. This paper describes the management of 2 girls with caudal duplication syndrome who underwent multistage reconstructive surgery. Both had a large omphalocele and a severe diastasis of the pubic symphysis. The first patient also had an apparent duplication of the vulva, the perineum and the anus to either side of a wide midline. Each vulva contained a urethra, a hemi-clitoris with ipsilateral labium minor, and a hemi-vagina with hemi-uterus. The second child had an infrapubic sequestrated appendico-cecal duplication lying between two hemi-bladders each with ipsilateral ureter and urethra. The everted duplication split the single vulva longitudinally in the midline as far as the fourchette. To each side were a hemi-clitoris, and a hemi-vagina with hemi-uterus and ipsilateral fallopian tube. Analysis of our patients' anatomy and a literature review indicates for the most part 'hemi' organs on either side and suggests that the term 'duplication' is a misnomer such that caudal 'split' syndrome may be a more appropriate title

Rare case of an adrenocortical neoplasm: A case report and review of literature

Adrenocortical neoplasms (ACNs) are rare and poorly characterized in infants. The true incidence of ACNs is not well known and it appears to vary substantially across different geographical areas. ACNs are more common in females and two peaks of incidence have been identified: The first year of life and between the age of nine and 16 years. Due to the heterogeneity and rarity of ACNs, their pathological and prognostic classification is challenging. The current study describes the case of a seven‑year‑old male, who presented to the Department of Pediatric Surgery, University of Siena (Siena, Italy) with a feminization syndrome and increased somatic growth that was associated with a unilateral adrenal mass, which was diagnosed by magnetic resonance imaging. Surgical excision of the mass was performed and histological analysis determined that it was an ACN, with a low risk of malignity; however, the pathological classification of the tumor was challenging. At present, the future behavior of ACNs is unpredictable. Therefore, increasing the knowledge surrounding this type of tumor may aid in its diagnosis, treatment and prognosis. Due to the rarity of pediatric ACNs, no single pediatric oncology center has acquired extensive experience treating this type of tumor. Thus, the initiation of an international tumor registry may aid with the management of patients presenting with ACN

ASSOCIATION BETWEEN DELETION 18Q AND TERMINAL DUPLICATION 1P IN PATIENT WITH BILATERAL VESICO-URETERAL REFLUX: CASE REPORT AND LITERATURE REVISION

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THE GASTRO-ESOPHAGEAL REFLUX IN CHILDREN IN VIDEOLAPAROSCOPY. TECHNIQUES TO COMPARE AND LONG-TERM FOLLOW-UP

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A CASE OF APPENDIX CARCINOID IN A PEDIATRIC PATIENT. A SUBTILE CLINICAL PRESENTATION

The appendix carcinoid, although rare (with an incidence of 0,08%), is the more frequent gastrointestinal tumor in children and teen. In most cases these tumors cannot be diagnosed or they can be incidentally found during a surgical exploration for acute abdomen. Only in few cases there are symptoms of a carcinoid's syndrome (flushing, diarrhea and wheezing). In this work we present the manegement of a patient with an appendix carcinoid revealed during an appendicectomy performed for a peritonitis caused by a perforated appendicitis, underlining the importance of diagnosis and long term follow-up

management and outcome of herlyn werner wunderlich syndrome conservative treatment in pediatric age

Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare congenital mullerian anomaly consisting of uterus didelphys, hemivaginal septum, and unilateral renal agenesis. Reported data in pediatric age are rare. We report our case of HWWS

BILATERAL VESICO-URETERAL REFLUX IN PATIENT WITH CROSSED RENAL ECTOPIA AND FUSION TYPE A

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