Polythelia in a 13-year old girl.

We present a rare case of a 13-year old girl with a bilateral polythelia. We would like to draw attention to this particular mammary malformation.

Prevention of complications during reoperative thyroid surgery.

BACKGROUND: Thyroidectomy performed by an experienced surgeon is associated with a low incidence of recurrent laryngeal nerve injury and permanent hypoparathyroidism. During reoperative thyroid surgery there is a higher technical risk because detection and preservation of the recurrent laryngeal nerves and parathyroid glands are more difficult than in the primary surgery. AIM: Our retrospective cohort study was to assess short- and long-term complications associated with reoperative thyroid surgery in order to suggest a technical approach to lower the morbidity rate. MATERIALS AND METHODS: From January 2005 to September 2013, 745 patients underwent surgery for thyroid disease. Before surgery all patients underwent clinical examination, laboratory blood tests, hormonal assays, neck ultrasound, chest radiography and indirect laryngoscopy. Patients were followed up at 1, 3, 6 months and then annually after operation with hormonal assays, blood tests and neck ultrasound. RESULTS: Eighty (10.7%) out of 745 patients (mean-age= 52.5 years; age-range 18-80) underwent reoperative surgery for recurrent thyroid disease. The primary treatments were enucleoresection (11.2%), thyroid lobectomy(56,3%), thyroid lobectomy with isthmectomy(10%) and subtotal thyroidectomy (22,5%). In the reoperative surgery group (Group Re) the transient RLN complications were 1.3% compared to 0.2% in the primary surgery group (Group P) (p= 0.51). The incidence of temporary hypocalcemia was 45% in the reoperative surgery group vs. 42.7% in the primary surgery group (p=0.72). CONCLUSIONS: Reoperative surgery should be reserved to experienced surgeons. However, even in this case, when surgical maneuvers reserved for primary surgery are applied, then this surgery is associated with a low complications rate.

Gastrointestinal stromal tumors treatment in the Imatinib era. The role of fair indication.

AIM: Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor of the gastrointestinal tract. Surgery remains the elective treatment. We retrospectively compared two group of patients, who underwent surgery for GIST before and after Imatinib advent in order to analyze the recurrence and survival rate. METHODS: Two patient groups who underwent surgery for GIST, from January 1997 to December 2002 (Pre-Imatinib group) and from January 2003 to December 2008 (Post-Imatinib group) were compared. Patients were evaluated on the base of gender, age, clinical manifestations, primary location and metastasis positivity, tumor size, mitotic index, immunoreactivity for CD117 and the outcome, including date of death. RESULTS: In the Pre-IM group only one patient died for prostate cancer, 12 months after operation, the other died because of GIST with a 24.6 months of median survival rate (range 15-51). In the remaining 12 patients the median follow up period was 55 months (range 6-152 months). In the Post-IM group the mean follow up was 50.7 months (range 26-74) and they are still being assessed for oncological as well as surgical treatment. CONCLUSION: Early diagnosis and radical resection remain the standard of cure for GISTs. To date, the use of Imatinib lead to its utilization as adjuvant and neo-adjuvant therapy in adults. Our experience suggests that there is a correlation between the mutational status of KIT and clinical outcome. These aspects should be explored for targeted therapy that can effectively combine biological therapy to surgery.

Valproic acid and phenobarbital blood levels during the first month of treatment with the ketogenic diet.

OBJECTIVE: The aim of this study was to assess how the ketogenic diet influences the blood levels of antiepileptic drugs in the first month of treatment in a pediatric population with drug-resistant epilepsy. METHODS: The plasma concentrations of antiepileptic drugs were investigated in an open study on 36 consecutive children and adolescents (20 males), aged between 6 months and 16 years (mean age 4.7 years), who were put on the ketogenic diet because of medically refractory epilepsy. The plasma levels of antiepileptic drugs were determined 30 days and immediately before the diet and on days 8, 15, 22 and 29 after the start of the diet. The daily dose of each drug was not changed during the first month of treatment, while the daily dose of benzodiazepines was reduced by up to 30% if excessive sedation or drowsiness occurred. RESULTS: While plasma concentrations of phenobarbital did not change in the first month on the ketogenic diet (mean increase of 2.3 mg/l ± 1.0), valproic acid showed a slight but not significant decrease (mean decrease of 6.7 mg/l ± 3.2), 2 weeks after the start of the diet. CONCLUSIONS: Adjustments in the daily dose of either drug before the start of the diet do not however appear to be justified.

[Incisional hernia prosthetic surgery: a prospective study comparing laparoscopic and open techniques]. / La chirurgia con protesi dei laparoceli: studio prospettico e di confronto tra le procedure laparoscopica e laparotomica.

BACKGROUND: Incisional hernia is a common complication of abdominal surgery and it is often a source of long-term morbidity. Surgical treatment include many different techniques and up to today the choice may be difficult. The employment of prosthetic materials has contributed to a remarkable improvement in the results of this surgery. We performed a prospective study to compare the outcomes after laparoscopic and open incisional hernia repair. PATIENTS AND METHODS: A total of 60 patients were assigned at random to two groups to be operated for median incisional hernia with mesh. Of these, 30 underwent laparoscopic repair and 30 open repair performed by Rives-Stoppa technique. Early and long-term outcomes were analyzed by a median follow-up of 45 months (range 31-78). Statistical analysis was done to asses differences between the groups. RESULTS: Both groups were homogeneus in terms of patient age, sex, body mass index, American Society of Anesthesiologists score and incisional hernia features. Laparoscopic repair is associated with a significantly lower incidence of wound infections, shorter operative time and hospitalization and faster return to work. The recurrence rate was similar between the two groups. CONCLUSION: In our experience laparoscopic incisional hernia repair appear to be a safe, feasible and effective alternative to Rives-Stoppa technique.

[Reoperative thyroid surgery: personal experience and review of the literature]. / Reinterventi in chirurgia tiroidea: contributo casistico e revisione della letteratura.

The goal of this study was to evaluate the complication rate of reoperative thyroid surgery and to find out the way to minimize the morbidity associated with it. We reviewed our experience in 622 patients, who underwent thyroid operation from January 2000 to September 2007. Among these ones, 76 were the patients who underwent reoperative thyroid surgery. Prior surgery in the 76 reoperations was: nucleo-resection in 9 pts (12.9%), lobectomy in 43 pts (55.5%), lobectomy+isthmectomy in 7 pts (9.3%), subtotal thyroidectomy in 17 pts (22.2%). Histologic examination revealed: benign lesions in 67 pts (88.15%), papillary cancer in 4 pts (5.26%), follicular cancer in 2 pts (2.63%), follicular adenoma in 1 pt (1.32%) and Hashimoto thyroiditis in 2 pts (2.63%). Complications included: section of recurrent laryngeal nerve, that was reconstructed in the same operation, and bilateral palsy of the recurrent laryngeal nerve so that was necessary to make a tracheotomy. One of the patient at the first thyroid surgery had monolateral palsy of the recurrent laryngeal nerve with dyspnoea and dysphonia. Temporary hypoparathyroidism (Ca<8 mg/dl) occurred in 47.3% of the patients, who underwent reoperative thyroid surgery and in 45.2% of the patients, who underwent prior thyroid surgery. Conclusions. This study documents that reoperative thyroid surgery can be performed with little morbidity to the patient if precise operative rules are respected.

[Extraperitoneal rectal cancer surgery]. / La chirurgia del carcinoma del retto extraperitoneale

Despite recent advances in radiation and chemotherapy, surgical resection remains the only potentially curative procedure for rectal cancer. The introduction of total mesorectal excision with autonomic pelvic nerve sparing and new modalities in restoring bowel continuity has improved significantly the prognosis as well as life quality of rectal cancer patients. Better results will be achieved only with a correct multidisciplinary approach. The Authors report their experience with surgical treatment of extraperitoneal rectal cancer, examine some important technical innovation and emphasize the oncological principles of radical surgery.

[Adenocarcinoma following restorative proctocolectomy for ulcerative colitis: a case report and review of the literature]. / Adenocarcinoma insorto dopo proctocolectomia restaurativa per rettocolite ulcerosa: presentazione di un caso clinico e revisione della letteratura.

Restorative proctocolectomy with ileal pouch-anal anastomosis has been accepted as the surgical treatment of choice for most patients with ulcerative colitis. The occurrence of adenocarcinoma arising near or into the ileal pouch is rare. Only 19 such cases have been reported so far. The authors report a case of a 67-year old male who developed an adenocarcinoma in the small rectal stump 12 years after a restorative proctocolectomy with double stapled ileal pouch-low rectal anastomosis for ulcerative colitis unresponsive to medical treatment. They, after a literature review, examine same steps of the procedure and emphasize the importance of regular and prolonged follow-up for all patients having restorative proctocolectomy for ulcerative colitis.

[Multiple repetitive mammary fibroadenomas. Case report]. / I fibroadenomi multipli recidivi della mammella. Case report.

Multiple and ripetitive mammary fibroadenomas represent a rare disease. The etiology is uncertain. The diagnostic radiology is able to allow a correct diagnosis. The authors report a case of a woman 22 years old undergone a surgical treatment many times to remove multiple and ripetitive mammary fibroadenomas. The proposed management provides for periodic radiological exams; the surgical treatment is proposed only for the lesions with a rapid growth.

[Double ileo-ileal and ileo-cecocolic intussusception due to submucous lipoma: case report]. / Doppia invaginazione ileo-ileale e ileo-ciecocolica da lipoma sottomucoso del tenue: case report.

Intestinal intussusception is a rare pathology in adults and counts for approximately 5-16% of all invaginations, according to literature. We report the case of a 54 years-old male affected by a double ileo-ileal and ileocecocolic invagination secondary to a submucosal intestinal lipoma. Because of a bowel obstruction symptomathology, the diagnosis was based on abdomen X-rays, abdominal ultrasound and multislice CT scan that showed a probable pre-operative diagnosis of intestinal intussusception. The diagnosis of double invagination was confirmed intraoperatively and was therefore performed a right colectomy extended to a long tract of ileum. Opening the specimen demonstrated the evidence of a submucosal lipoma on the head of the intussusceptum. After the description of this case, we proceed with review of the literature of adult intestinal invagination.

[Adenocarcinoma of the appendix. A case report and review of the literature]. / L'adenocarcinoma dell'appendice ileo-ciecale: presentazione di un caso clinico e revisione della letteratura.

Primary adenocarcinoma of the appendix is a rare malignancy that constitutes less than 0.5% of all gastrointestinal neoplasms. Usually the diagnosis is made only after histological examination of surgically removed inflamed appendix. Alternatively represent an unexpected finding, confirmed by frozen section, during surgery performed for acute appendicitis or other non appendiceal pathologies. Natural history is strongly influenced by anatomic peculiarities of the appendix that predispose to early spread and perforation. Frequently is associated with synchronous and metachronous colorectal or extraintestinal cancers. The correct management is the right hemicolectomy as a primary procedure in the case of preoperatively or intraoperatively diagnosis or as secondary procedure, after two-three weeks from appendectomy, when the microscopic examination of specimen reveals the presence of adenocarcinoma. Right hemicolectomy is the best treatment for all histologic types (colonic, mucinous, adenocarcinoid), in presence of perforation and even in Dukes A tumors. A careful intraoperative search for synchronous lesions and a life-long program of surveillance for the detection of early stage metachronous carcinomas are recommended. The Authors report a case of primary adenocarcinoma of the appendix occurred in a 78 year-old female patient, diagnosed incidentally during surgery performed for ileus from suspected cecal neoplasm.

[Modern orientation in parotid surgery]. / Moderni orientamenti nella chirurgia della parotide.

The choice of the surgical approach is very relevant in parotid surgery because of the extreme anatomic variability of the parotideal district and the functional importance of the branches of facial nerve. The aim of this paper is to prove how an accurate knowledge of the anatomy and a careful execution of surgery permit radicality in case of tumors and respect of nerves and other structures of the region. We studied anatomy of the different intra- and periparotideal structures to compare two different approaches in parotidectomy, proving how the orthograde technique is safer and more effective in preventing hyatrogenous nerve injuries and other major complications.

[Mininvasive treatment of strangulated paraesophageal hiatal hernia in emergency. Case report]. / Trattamento chirurgico minivasivo d'urgenza di ernia gastrica iatale paraesofagea strozzata. Caso clinico.

The Authors report a case of strangulated paraesophageal hiatal hernia occurred in a elderly woman and treated with laparoscopic approach. After review of the literature regard on this uncommon pathology that present about 5% of the hiatal hernias, they emphasize that the laparoscopic approach is appropriated even in emergency and comprises complete reduction of the stomach in abdomen, control of suitable position of the distal esophagus and cardias and making of effective hiatus-plasty.

Involvement of norepinephrine in the control of activity and attentive processes in animal models of attention deficit hyperactivity disorder.

Functional and morphological studies in children affected by Attention Deficit Hyperactivity Disorder (ADHD) suggest a prefrontal cortex (PFc) dysfunction. This cortical region is regulated by subcortical systems including noradrenergic (NEergic), dopaminergic (DAergic), cholinergic, serotonergic, and histaminergic pathways. A wealth of data in humans and in animal models demonstrates altered dopamine (DA) regulation. Drugs that modulate norepinephrine (NE) transmission are also effective in ADHD patients, thus leading to the hypothesis of a NEergic disorder. This review covers the regulation of PFc functions by NE and the interaction between the NE and DA systems, as suggested by pharmacological, electrophysiological, morphological, and gene knock out (KO) studies. A negative feedback between NE and DA neurons emerges from KO studies because KO mice showing increased (NE transporter (NET) KO) or decreased (DBH and VMAT2 KO) NE levels are respectively associated with lower and higher DA levels. Locomotor activity can be generally predicted by the DA level, whereas sensitivity to amphetamines is by NE/DA balance. Some animal models of ADHD, such as spontaneously hypertensive rats (SHR), show alterations in the PFc and in the DA system. Evidence about a correlation between the NE system and hyper-locomotion activity in such animals has not yet been clarified. Therefore, this review also includes recent evidence on the behavioral effects of two NET blockers, reboxetine and atomoxetine, in two animal models of ADHD: SHR and Naples High Excitability rats. As these drugs modulate the DA level in the PFc, certain effects are likely to be due to a rebalanced DA system. We discuss the significance of the results for theories of ADHD and make suggestions for future experimentation.

A combined approach of neoadjuvant chemotherapy and surgery for colorectal liver metastases.

Hepatic metastases represent one of major clinical problems in patients affected by solid tumours because liver is the first site of metastatic disease after lymph nodes, (1, 2). The most common origins among adults are from colon and rectum cancer followed by pancreas (adenocarcinoma or neuroendocrine), lung and breast (3-7). It is estimated that in 15-20% of patients affected by adenocarcinoma of colon-rectum liver metastases are present at the time of diagnosis (8-11), while in 40% of cases they will develop during the follow-up (10, 11). Liver metastases are the first determinant for survival (1, 3, 12-17), and both hepatic and extrahepatic involvement have a statistical significant impact on it (3, 14, 18-21). Prognosis of patients affected by untreated disease is very poor, being 5 to 14 months with rare 2-year survivors and a 5-year survival rate of 0% (10, 21-24). In half of all patients affected by colorectal cancer and undergoing to a radical surgery, disease will fail in the liver (8, 25-28); among patients dying for colorectal cancer, 60-70% have a liver involvement (9, 29), and in 20-25% liver is the only site of recurrence and death results from liver failure (3, 29-31). In Autoptic studies those rates increased up to 83% and 38% respectively (1, 10, 17, 32).

Peritoneal mesothelioma in a case of inguinal hernia. A review of the literature.

The peritoneal mesothelioma (PM) is a rare, benign or malignant, primary tumour, arising from the peritoneal membrane. The most frequent histological form is the malignant one with an incidence of 2-2.6 new cases per million per year. The symptomatology is insidious and poses difficult problems in the diagnosis and the treatment. Instrumental diagnostic investigations are useful only in the diagnostic orientation. Only the pathologic examination allows to distinguish a peritoneal carcinomatosis from PM. The prognosis of MPM is pour. An intense multimodal therapy, combining surgery with CT and RT, increases the survival rates in the patients with MPM. It has been proposed that hernia of abdominal wall play a role in the pathogenesis of this tumor. We believe that hypothesis seems unlikely considering the enormous discrepancy between the incidence of hernial pathology and PM.

[Hereditary nonpolyposis colorectal cancer (Lynch syndrome): a review of the literature and case reports]. / Cancro colorettale ereditario non poliposico (sindrome di Lynch): revisione della letteratura e contributo casistico.

The recent observation of a new HNPCC patient case induced the Authors to review their experience with the syndrome as well as to make an up to date of the problems related to diagnosis, surgical management, surveillance and genetic counselling for such patients with a lifelong high cancer risk. Patients with HNPCC and their first-degree relatives, whose risk of early colorectal carcinoma (especially in the proximal colon) as well as a variety of extracolonic cancers (particularly endometrium, ovary, stomach, small bowel, ureter and renal pelvis) is significantly higher then that of patients with sporadic carcinoma, should be properly managed with surgery and then with endoscopic examination (ideally all life long) starting--in unaffected individuals--at early age (25 years old). Problems related to genetic counselling are considered as well.

[The megacolon in myotonic dystrophy: case report and review of the literature]. / Il megacolon nella distrofia miotonica: case report e review della letteratura.

Myotonic dystrophy (MD) is an autosomal dominant inherit disease, slowly progressive, involving multiple organ systems. Disorders at any level of the gastrointestinal tract are relatively common and manifest as disturbances in motility, such as impaired esophageal transport, delayed gastric emptying, and megacolon. A 51 years-old man was admitted to our surgical department with obstructive symptoms. Diagnostic evaluation showed megacolon and the typical clinical features of the MD, such as weakness, myotonia, frontal baldness and testicular atrophy. Risk of perforation and dehydration led to emergency total colectomy with ileorectal stapled anastomosis. The patient didn't suffer for compliance related to surgical treatment but, after 1 month in intensive care, died of pneumonia and myocardial infarct. The overall frequency of perioperative complications in patients with MD ranges from 8.2 to 42.9%. The risk of perioperative pulmonary complications is particularly high. Thus, we believe that the conservative treatment of motility disorders of the bowel in patients with MD is to be justified and that surgical treatment should be reserved, as last resort, performing a early diagnosis and careful monitoring during perioperative period.

[Synchronous and metachronous colorectal cancers: a case series contribution]. / I cancri sincroni e metacroni del colon-retto: contributo casistico.

The Authors, on the basis of their experience with neoplastic colorectal pathology and after a review of the Literature, report a reappraisal of the problems related to colorectal multiple carcinomas. They emphasize the importance of routine preoperative pancolonoscopy for the identification of possible synchronous tumors (both benign and malignant) and periodic endoscopic follow-up (ideally a life-long one) for the detection and removal of all adenomatous polyps as well as early stage metachronous carcinomas, especially for patients with HNPCC. Besides, they stress the importance of sensibilization of the population about the heritability of colorectal carcinomas.