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Congenital prepubic sinus (CPS) is a rare congenital variant of urethral duplication, with less than 50 cases documented in the scientific literature since it was first reported by Campbell et al., in 1987. An 8-year-old male patient complained of an inflammatory secretion from a sinus opening on the dorsal base of his penis. Physical examination revealed an accessory orifice in the midline of the dorsal proximal penis without any penile curvature. An ultrasound (US) examination found an 8-mm collection of cutaneous and subcutaneous tissue at the dorsal base of the penis. The renal US, however, showed no abnormalities. Additionally, micturating cystourethrogram (MCUG), was negative for vesicoureteral reflux (VUR). Retrograde sinusography (RS) and urethrogram showed no communication between the urinary tract, and the sinus was found to end blindly at the level of the pubic symphysis. A surgical excision was decided, to avoid potential future complications. A 3-cm-long intact sinus was dissected and excised entirely. Histological examination confirmed the diagnosis of a urethral duplication. The postoperative course was uneventful. To date, at six-month of follow-up, the patient remains asymptomatic. The histological findings in this case support the theory that CPS is a variant of dorsal urethra. Urethral duplication should always be suspected in the case of accessory dorsal openings. Surgical excision is recommended at an early age, even in the absence of symptoms, to improve aesthetics, prevent recurrent infections and avoid the development of future malignant tumors. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Levodopa is the gold standard in the pharmacological treatment of Parkinson’s disease (PD) and its oral administration is associated with the development of disabling motor and non-motor complications in advanced disease. Levodopa is rapidly metabolized and has a short plasma half-life thus requiring frequent, repeated dosing. Impaired gastric emptying is common in PD, and likely contributes to the unpredictable motor responses observed with orally-dosed levodopa. A new therapeutic protocol for patients with advanced PD include a carbidopa/levodopa combination using continuous, modulated enteral administration achieved inserting a Jejunal Extension Tube Placement through Percutaneous Endoscopic Gastrostomy (PEG-J). The aim of this work is to assess efficacy and safety of levodopa-carbidopa intestinal gel (LCIG) delivered continuously through an intrajejunal percutaneous tube (PEG-J). PATIENTS AND METHODS: We enrolled 11 adults with advanced PD and preserved sensitivity to L-dopa. For pre-procedural endoscopic evaluation each patient underwent a diagnostic esophagogastroduodenoscopy (EGD) 7 days before PEG-J placement to evaluate the presence of gastric anatomical or wall anomalies and the presence of oesophageal or gastric varices. Treatment with LCIG, consisting of a waterbased suspension containing micronized levodopa (20 mg/mL) and carbidopa (5 mg/mL) in methylcellulose (Duodopa®), was administered by continuous jejunal infusion for 12h/day using a portable pump (CADD-Legacy) by PEG-J. Clinical evaluations were performed at baseline (T0) before LCIG initiation, and after 3 (T3) and 6 (T6) months of therapy. The efficacy and safety out-comes were assessed by using the Unified Parkinson’s Disease Rating Scale (UPDRS) parts II, III and IV. RESULTS: Mean age of patients was 71.18 ± 5.4 SD at LCIG initiation. Out of the 11 patients, 2 (18%) dropped-out LCIG at T3. Patients showed statistically significant (p < 0.05) higher performances in activities of daily living and a statistically significant (p < 0.001) lower incidence and severity of motor fluctuations, as rating by UPDRS part IV, compared to their best oral therapy. During observational period, 5 patients experienced adverse events. Success rate for PEG-J placement was 100%. CONCLUSIONS: Our work shows that continuous intrajejunal infusion of LCIG ensures a reduction in motor Fluctuations compared to oral administration of levodopa-carbidopa in advanced PD. Based on our results and on the evidence emerging in the literature, this therapeutic approach should be the gold standard for therapy in these patients. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Sclerosing mesenteritis (SM) is a rare, idiopathic disorder of unknown aetiology that involves the adipose tissue of the mesentery, being characterized by chronic and non-specific fibrous inflammation. Patients usually present with non-specific clinical manifestations, such as abdominal pain and diarrhoea. The diagnosis of SM is difficult and it can be definitely established only by means of surgical or imaging-guided biopsy. Different therapeutic strategies have been used in case series with different rate of success. The disease is generally self-limiting, and the long-term prognosis is good, even if some cases of severe SM are reported in literature. Here, we report a fatal case of sclerosing mesenteritis associated to protein-losing enteropathy. [ABSTRACT FROM AUTHOR]

The management of choledochal cysts has evolved during the last 3 decades. Laparoscopic surgery has revolutionized the treatment of choledochal cyst (CC). We report our case, 2-year old female, in which total excision of cyst and Roux-en-Y hepaticoenterostomy is done by laparoscopic approach. In the hands of experts, laparoscopic excision of the CC is safe and effective approach. This is our first experience and our outcome has been good to a follow-up of 6 months. In our opinion, laparoscopic surgery is a safe treatment of choledochal cyst with less postoperative morbidity, a shorter length of stay and a lower blood loss when compared with open approach. Laparoscopic surgery may become the first choice procedure for choledochal cyst. [ABSTRACT FROM AUTHOR]

Hypertrophic pyloric stenosis (HPS) is the most important cause of non-bilious vomiting during infancy and it is usually idiopathic. It is very rare in older children and adolescents, in which gastric outlet obstruction (GOO) is more typically secondary to other conditions. Gastrointestinal malformation are frequently associated with Down syndrome and some of them (especially duodenal abnormalities) can be detected quite late. In infants the standard treatment of HPS is represented by a Ramstedt pyloromyotomy, while in adults a distal gastric resection is more indicated and it is usually associated to a Billroth I or II reconstruction. We present an unusual case of a 12-year-old girl with Down syndrome, diagnosed with late-onset HPS. She came to our attention with periodic non-bilious vomiting and growth delay. She underwent multiple imaging examinations, which highlighted the presence of an HPS. Based on patient's age and the thickness of the pyloric muscle, we decided to perform a partial gastrectomy with a Roux-en-y reconstruction. Patient was discharged on the X day. She is now on follow up, gains weight and presents good conditions. HPS is rare but possible during childhood and adolescence. Diagnosis is usually performed through ultrasounds (US) and upper gastrointestinal radiological series (UGI), but sometimes further examinations are required (abdominal MRI, esophagogastroduodenoscopy). In older children since less invasive techniques are not effective, we have to consider partial gastrectomy. Based on our experience, Roux-en-y reconstruction is a valid option, associated with poor complication and good quality of life. [ABSTRACT FROM AUTHOR]

Juvenile myasthenia gravis (JMG) is a rare debilitating and potentially fatal autoimmune disease, with unclearify pathogenesis. Surgical immunomodulation with thymectomy has been repeatedly demonstrated to be a safe and effective treatment for JMG in both adult and pediatric patients. In the last few years, minimally invasive approach and above all robotic assisted thoracoscopy, replaced sternotomy which is widely used in adult patients. We report our experience in a case of robot-assisted thoracoscopic thymectomy approach in a 12 years old boy for juvenile myasthenia gravis (JMG). Procedure was performed with the Da Vinci surgical robot (Xi) using left-sided approach. Left lung was kept out from mechanic ventilation. A 8 mm port for the 3-D camera was introduced on the V intercostal space on the anterior axillary line. Others two 8mm thoracic ports were placed under vision after the induction of a low-pressure pneumothorax, respectively on the midaxillary line on the III intercostal space and on the parasternal space on the V intercostal space. Bulk resection of gland was made using Maryland grasper and Harmonic scalpel, starting at level of the left pericardiophrenic angle and continuing cranially. Thymus was unstick from the posterior face of the sternum until the right pleura releasing lower thymic horns. After that, controlateral right-side thymectomy was continued into the neck to include the upper horns and finally it was removed with an endocatch bag. Histopathological examination showed a benign thymic hyperplasia. There were no perioperative and postoperative complications. The discharge was on IV post-operative day. After thymectomy, patient reported an improvement in symptoms and stopped medical therapy with pyridostigmine. VATS and Robotic thoracoscopic thymectomy have increasingly taken hold in recent years. The surgical treatment offered to patient an improvement in clinical status. Surgery by robotic assistance has demonstrable advantages, including three-dimensional visualization and articulating instruments. Left lateral approach provided excellent visualization of the thymic veins, anonymous vases and phrenic nerves. Three-dimensional visualization as well as articulating arms greatly facilitated the dissection compared with standard thoracoscopic technique. In Literature very few series of robotic approach for surgical treatment of JMG in children are reported, for these reasons further studies are needed. [ABSTRACT FROM AUTHOR]

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