Michela Maffi

Long-term follow-up after esophageal replacement in children: 45-Year single-center experience

PURPOSE We evaluated the results of esophagocoloplasty (ECP) in children by performing a 45-year retrospective cohort study in a single center. MATERIALS AND METHODS We collected all of the medical charts of patients who underwent ECP at our hospital from January 1969 to January 2014. We reviewed the medical history for each patient and then contacted the patients by phone to obtain responses to a questionnaire. The questionnaire evaluated the following four areas: overall general state, gastrointestinal function, dependence on the Health Sanitary System, and their aesthetic satisfaction. RESULTS We contacted 96 patients, and 72 completed our questionnaire. There were 45 males and 27 female respondents, with a mean age of 34.5 years. The mean Karnofsky performance status index was 96.4%. There were excellent scores obtained from 55 patients and fairly good scores from 16 patients. There was 1 patient with a low score, and there were no scores reported below 70%. Most patients (58/72) reported regular bowel habits, and no patients required drugs or other measures to facilitate defecation. Gastroesophageal reflux was present in 51 patients, and 62.8% have bothersome symptoms. Twenty-five patients used pharmacological therapies. Forty-eight patients (66.6%) were satisfied with the aesthetic result of surgery (mean score in a scale from 0 to 5, is 3). CONCLUSIONS ECP can be used in children who require esophageal substitution. The resulting long-term QoL is acceptable. However, the aesthetic outcomes remain a problem.

Ovarian Tissue Collection for Cryopreservation in Pediatric Age: Laparoscopic Technical Tips

As the number of young girls subjected to chemo and radiotherapy is steadily increasing it is our duty to try to preserve their future fertility and ovarian function. A possibility is to collect ovarian samples, cryopreserve them and reimplant the tissue when the treatments are over. We report our 11 year experience with the use of laparoscopy to collect ovarian tissue samples. In particular we focus on the details of the surgical technique. The procedure was performed in 54 patients affected by malignancies or pathologies requiring chemo or radiotherapy, with good results. Cryopreservation seems to be a good option to preserve future fertility although it is still an experimental method.

Laparoscopic resection of a rare gastrointestinal stromal tumor in children

IntroductionGastrointestinal stromal tumors (GIST) are uncommon mesenchymal tumors of the gastrointestinal (GI) tract, accounting for 0.1% to 3% of all GI malignancies. Paediatric GIST have an annual incidence of 0.02 per million children, have a female predisposition, are usually located in the stomach (50–60%) and in up to 85% of cases CD117-cKit or PDGFRA mutation is absent, resulting in a decreased efficacy of the target therapy.Case descriptionWe report the case of an incidentally diagnosed gastric GIST in a 14-year-old boy with multiple malformations. Genetic tests and Kariotype resulted negative.Recently, an abdominal US visualized an hypoechoic heterogeneous abdominal mass. The common tumor markers resulted negative and the abdominal CT-scan confirmed the presence of a solid round lesion (42×36mm) in contact with the stomach and the pancreas. Laparoscopy allowed the recognition and the removal of the nodular mass at the posterior margin of the stomach. The histopathologic and the molecular biology findings were consistent with a kit-wilde type GIST. Surgical margins were microscopically free of tumor cells.These results justify the decision not to add other surgical or medical therapy. However, for high risk of recurrence and metastasis, a close follow-up was started.Discussion and evaluationGIST are asymptomatic in 10% to 30% of patients or present nonspecific symptoms and signs. These tumors present usually irregular, lobulated and ulcerated. CT-scan of the abdomen and pelvis or magnetic resonance imaging (MRI) are mandatory in the diagnostic work-up. The final diagnosis is based on histology and immunohistochemistry. Surgery is the first-line treatment in patients with localized disease.ConclusionGuidelines for the management of pediatric GIST are not presently available for the paucity of reports and data. However it is widely accepted that surgery is the first-line treatment and gross resection with negative microscopic margins can be considered therapeutic and lead to full remission of the pathology. Laparoscopy is a safe surgical approach for the exploration of the abdominal cavity, the evaluation of the disease and the complete removal of the tumor.

An Interesting Case of Double Compound Intussusception without Intestinal Occlusion in a 5-Year-Old Boy

Intussusception is a very common surgical finding in children. Idiopathic intussusception is relatively frequent in children within the first year and it usually causes an acute abdomen. Instead, complex intussusception involving multiple or remote intestinal segments is a very rare entity and it is frequently diagnosed intraoperatively. It is often because of an anatomical cause and it can occur at any age. We report a unique case of contemporary double site anterograde and retrograde ileoileal intussusception without intestinal occlusion because of a submucous intestinal lipoma. In our case, imaging studies were important for clinical suspicion and laparoscopy was essential for final diagnosis and its resolution.

A Rare Case of Chylothorax due to Pulmonary Lymphangiectasia in a 7-Year-Old Boy.

Pulmonary lymphangiectasia (PL) is a rare condition characterized by dilatation of the lymphatic vessels. Post-neonatal PL is usually associated with pleural effusion and should therefore be suspected in the presence of chylothorax. We describe a post-neonatal manifestation of PL in a 7-year-old boy presenting chylothorax. Radiological examinations included thorax X-ray, ultrasound, and computed tomography scans. After the failure of conservative management (maintenance of the chest tube, total parenteral nutrition, administration of somatostatin synthetic analogues) we performed a thoracoscopic massive ligation of the thoracic ducts collateral along with a lung biopsy. Histology was compatible with type 1 congenital pulmonary lymphangectasia. One month after surgery a thoracoscopic pleurodesis was required for persistent chylothorax. The boy is now doing well 1 year after surgery.

Secondary Omental Torsion as a Rare Cause of Acute Abdomen in a Child and the Advantages of Laparoscopic Approach

Omental torsion is an underdiagnosed cause of acute abdomen in children. Most cases occur in adults, with only 15% presenting in children. It is estimated that omental infarct is found in approximately 0.1 to 0.5% of children undergoing operations for appendicitis. It may present with various signs and symptoms, although the clinical presentation usually mimics that of acute appendicitis. Definitive diagnosis is often established during surgery. We report a case of a 9-year-old boy who came to our attention for acute abdomen. We performed a laparoscopy-assisted procedure suspecting acute appendicitis. We therefore identified a secondary omental torsion associated with patency of the right inguinal duct and herniated omentum. Laparoscopy allowed a correct diagnosis and therapy.

Robotic removal of Müllerian duct remnants in pediatric patients: our experience and a review of the literature

Persistent Müllerian duct syndrome is a disorder of sexual development, which features a failure of involution of Müllerian structures. An enlarged prostatic utricle is a kind of Müllerian duct remnant (MDR) with a tubular shaped structure communicating with the prostatic urethra. Treatment is aimed at relieving symptoms when present, preserve fertility and prevent neoplastic degeneration. We describe 3 cases of successful robot assisted-removal of symptomatic MDRs. The first case came to our attention for pseudo-incontinence; the other two for recurrent urinary tract infections. The patients have not presented such symptoms anymore on follow-up. We then reviewed existent literature on authors who have recently investigated the main issues concerning MDRs and have attempted a roboticassisted approach on them. Robot-assisted laparoscopy can be considered a valid, safe and effective minimally-invasive technique for the primary treatment of prostatic utricle.

Robot-assisted Pyeloureterostomy in Bifid Renal Pelvis With Ureteropelvic Junction Obstruction of the Lower Moiety

Duplications of the urinary collecting system and pyelo-ureteral junction obstruction (PUJO) are common, but the simultaneous presence of both anomalies is rarely encountered. In incomplete duplicated systems, PUJO usually affects the lower moiety. We present the case of a 5-year old boy with left bifid renal pelvis and hydronephosis of the lower moiety treated by robot-assisted pyeloureterostomy.

Shifting from Conventional Minimally Invasive Surgery to Robotic Surgery

Robotic surgery has been introduced into clinical practice in the late 1990s to overcome well-recognized limitations of the conventional minimally invasive approach, including two-dimensional imaging, restricted range of motion of the instruments, hand tremors, and poor ergonomic positioning of the surgeon. Since then, robotic surgical systems have rapidly evolved and are used for an increasing number of complex minimally invasive surgical procedures [1–6]. Historically, new surgical techniques have had a more difficult and slower acceptance by the pediatric surgical community compared to the adults’ one. Indeed, small spaces and anesthesiological management have limited the use of these technologies. In the pediatric age, robotic surgery has been accepted and utilized by a small number of pediatric surgeons around the world. Since the first reported case in a child in April 2001, the use of robotic technology has rapidly expanded within pediatric surgery. During the last decade, it has successfully been applied to a large variety of gastrointestinal, genitourinary, and thoracic procedures in infants and children, thus demonstrating the safety and feasibility of this approach. The number of pediatric robotic procedures performed per year using this emerging method is growing rapidly, with no evidence that this will change in the future. The early functional outcomes of robotic procedures are promising; however at present, most of the comparative studies are from single institutions, and lack a high level of evidence. Although increasing numbers of larger pediatric robotic surgery case series have been published over the years, authors mainly focused on the comparison with open surgery. However, in order to identify potential advantages of a particular type of robotic procedure over the corresponding conventional laparoscopic or thoracoscopic approach, comparative studies providing evidence-based information are needed [6–10]. Performing robotic surgery in pediatric patients requires a complete new redesigned concept of the surgical techniques and modifications in the surgical operating room. The introduction of this innovative technology brings new advances in instrumental maneuverability, and better optics. However, robotic surgery also brings new challenges and limitations that will require improvement in the future. The successful transition from laparoscopy to robotic surgery requires some steps. These include developing a specific robotic team that should be well informed on setting up the robot and can deal with intraoperative problems. Another task of the team is to ensure that the surgeon has spent sufficient time on the robot to be familiar and proficient, and to help in the selection of patients, thus avoiding difficult cases in the beginning. During the first procedures it is important to have sufficient time so that no one is rushed or harassed by lack of time, and, of course, to make sure that a proctor is present. Finally, when the surgical session is over, a debriefing with the whole team will minimize problems, develop enthusiasm for this new technology, and allow checklists and protocols to be developed. With these simple steps, this transition can be performed relatively painlessly [11].

Portal Hypertension in Children

Portal hypertension is defined as an increase of blood pressure in the portal venous bed beyond the physiological values.