Alfredo Garzi

Association of microphthalmia and esophageal atresia: Description of a patient and review of the literature

Since 1988, when Rogers first described a boy with anophthalmia associated with esophageal atresia, eight similar cases have been reported. These patients lend support to the hypothesis that this association of congenital anomalies constitutes a discrete entity, although the etiology is still unknown.We report a patient with this combination of malformations as well as a marked hypoplasia of the entire left half of the body.

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.

Minimally invasive surgery for achalasia: combined experience of two European centers.

Background: The present report summarizes 12 cases with achalasia treated with laparoscopic Heller myotomy and anterior fundoplication according to the method of Thal and Dor. Patients and Methods: From 1997 to 2005 at 2 institutions in Europe, 12 patients (7 male and 5 female, ages ranging from 3.5 to 7 years) were treated for esophageal achalasia (EA) with laparoscopic Heller myotomy and anterior fundoplication according to Thal and Dor. In 1 case a perforation of the esophageal mucosa occurred, which was promptly treated during surgery. Follow-up ranged from 18 to 60 months and included clinical examination and barium radiography of the upper digestive tract. Results: Postoperatively, 10 patients showed a normal weight curve and complete resolution of symptoms. All patients showed complete regression of digestive and respiratory symptoms from the first examination, with a normal oral meal intake and an improvement of weight and height parameters. One case required repeat intervention after 2 years because of persisting pain; in this case surgery revealed an insufficient myotomy. Conclusions: Modified extramucosal Heller cardiomyotomy associated with 180° anterior antireflux plastic surgery (according to Thal and Dors procedures) is a useful and safe procedure in the treatment of EA in pediatric patients. Our data, which are supported by long-term follow-up, also stress the relevance of anterior fundoplication in preventing postoperative gastroesophageal reflux.

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.

A Rare Case of Neonatal Ileo-Cecal Valve Stenosis due to Covered Iliac Perforation

At birth, newborn babies tend to respond to any stressful event with elective blood shunting towards the main organ systems, such as the brain and heart. Consequently, the bowel may suffer from a severe flow reduction with a high risk of hypoperfusion. The distal ileum is relatively less vascularized than other parts of intestine, due to a lack of collateral arteriolar circulation. It is therefore at higher risk of hypoxia, tissue necrosis and perforation in situations of minimal hypoperfusion or spasm. A rare case of covered perforation of the last loop of the ileum in a severely preterm baby is reported. The perforation manifested atypically as inflammatory stenosis of the loop and the ileo-cecal valve. To avoid misdiagnoses and to manage these cases correctly, the caregiver has to consider this rare eventuality in the differential diagnosis of intestinal occlusion/subocclusion in the first days of life.

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.

Prenatal diagnosis, 3-D virtual rendering and lung sparing surgery by ligasure device in a baby with “CCAM and intralobar pulmonary sequestration”

Abstract Congenital cystic lung lesions are a rare but clinically significant group of anomalies, including congenital cystic adenomatoid malformation (CCAM), pulmonary sequestration, congenital lobar emphysema (CLE) and bronchogenic cysts. Despite the knowledge of these lesions increasing in the last years, some aspects are still debated and controversial. The diagnosis is certainly one aspect which underwent many changes in the last 15 years due to the improvement of antenatal scan and the introduction of 3-D reconstruction techniques. As it is known, a prompt diagnosis has an essential role in the management of these children. The new imaging studies as 3D Volume rendering system are the focus of this paper. We describe our preliminary experience in a case of hybrid lung lesion, which we approached by thoracoscopy after a preoperative study with 3D VR reconstruction. Our final balance is absolutely positive.

Rare Surgical Emergencies

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 centers have changed the clinical approach to these kinds of emergencies. In fact, most conditions, leading to gastrointestinal (GI) emergencies, are uncommon and treatment in specialist centers enables concentration of appropriate resources and expertise. Co-morbidity is common, particularly in the preterm or low birth weight infant [1].