E. Bindi

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.

Juvenile granulosa cell tumor of the testis: prenatal diagnosis and prescrotal approach.

Neonatal testicular tumors are rare and should be considered in the differential diagnosis of newborn scrotal masses. Juvenile granulosa cell tumor (JGCT) accounts for about 5% of all prepubertal testis tumors. As a benign neoplasm, radical orchiectomy is sufficient for treatment. We report a case of a newborn with a prenatal diagnosis of scrotal mass. After surgery, the histological diagnosis was juvenile granulosa cell tumor. To date the patient is healthy.

Persistent Nonbilious Vomiting in a Child: Possible Duodenal Webbing

An association between malrotation and congenital duodenal webbing is rare. We present our experience with four patients at two centers, and a review of published reports. There are currently 94 reported cases of duodenal pathology associated with malrotation. However, only 15 of the 94 cases (15.9%) include patients with malrotation and a duodenal web. We suggest that nonbilious vomiting in a child must prompt the surgeon to consider duodenal pathology even in the presence of malrotation.

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.

Preputialplasty: can be considered an alternative to circumcision? When, how, why? Experience of Italian centre

Background Phimosis is a condition in which the foreskin cannot be portrayed on the glans. It is a physiological and common condition in the pediatric age. The pathological form derives from an inflammatory or traumatic lesion. Circumcision is the most common surgical treatment of phimosis but it is a controversial practice, especially in occidental world. Methods We enrolled 61 patients with pathological phimosis (22/balanoposthitis, 18/painful erection, 21/urinary discomfort) between 2015-2017. Results All patients underwent preputialplasty. Conclusions Various alternatives to circumcision have been described, as manual retraction therapy, topical steroid therapy, and many types of preputialplasty. We report our technique.

Pediatric Presentation of a Celiaco-mesenteric Trunk in Association with a Pelvic Bilateral Renal Ectopia: An Undescribed Association

Background: The celiac trunk presents its normal trifurcation in the 87% of cases reported in autoptic and radiological studies. Among variants the anatomical conformation known as celiaco-mesenteric trunk has an incidence of 1-2.8%. Case Report: We hereby report a 6 years old girl who presented with recurrent abdominal pain and bilious vomiting. MRI and CT scan, performed with the diagnostic suspicion of an aorto-mesenteric compass, showed the presence of a common origin of the celiac trunk and superior mesenteric artery. In addition, images revealed also the presence of a bilateral pelvic ectopia of kidneys, with renal arteries which took origin from iliac bifurcation. The patient, two months after, was submitted to surgery, during which we found the presence of an intestinal malrotation, instead of an aorto-mesenteric compass. Conclusion: An adequate evaluation of radiologic images and the use of 3D reconstruction permits to diagnose anatomic variants of celiac-mesentric artery.

Isolated Vaginal Agenesis Associated with Multiple Gastrointestinal Anomalies: A Case Report

More than 50% of infants with esophageal atresia have associated anomalies. We present a case report of a 46XX neonate with long-gap esophageal atresia and tracheoesophageal fistula (EA/TEF), anorectal malformation, bowel duplication and vaginal agenesis. This is an unusual association of abnormalities which had not yet described in literature.