Vitamin D and Bone Metabolism in Adult Patients with Neurofibromatosis Type 1.

Neurofibromatosis type 1 (NF1) is a genetic multisystemic autosomal dominant disorder determining reduced life expectancy due to higher risk of developing benign and malignant tumors. Low levels of vitamin D and reduced bone mineral density (BMD) have been reported in young patients with NF1. However, correlation between vitamin D and NF1 phenotype needs to be elucidated. Aim of this study was to assess vitamin D levels and bone metabolism in NF1 patients, analyzing potential correlations with clinical phenotype. A cross-sectional study was carried out in a monocentric series of NF1 patients, evaluating genotype, clinical phenotype, BMD, biochemical evaluation with focus on serum 25OH-vitamin D, parathyroid hormone (PTH), calcium and phosphate levels. Correlations between clinical manifestations, neurofibromas, and vitamin D status have been studied in comparison with healthy controls. 31 NF1 adult patients were matched for sex, age and body mass index with 31 healthy controls. A significantly difference in vitamin D level emerged in NF1 patients compared to controls. Interestingly low vitamin D levels correlated with a more aggressive phenotype and with a bigger size of neurofibromas. These data underline that vitamin D deficiency/insufficiency may play a role in clinical severity of neurofibromas in patients with NF1, suggesting the need to check bone status and replace vitamin D in these patients.

Complete Fundus Mobilization Reduces Dysphagia After Nissen Procedure.

Background. Anti-reflux surgery is an effective treatment for gastroesophageal reflux disease (GERD). Nevertheless, surgery is still indicated with great caution in relation to the risk of complications, and in particular to postoperative dysphagia (PD). Objective. To compare the clinical outcomes, with particular focus on the incidence and severity of PD, of laparoscopic Nissen-Rossetti fundoplication (NRF) and floppy Nissen fundoplication (FNF) with complete fundus mobilization, in the surgical treatment of GERD. Methods. Ninety patients with GERD were enrolled. Forty-four patients (21[47.7%] men, 23[52.2%] women; mean age 42.4 ± 14.3 years) underwent NRF (Group A), and 46 patients (23[50%] men, 23[50%] women; mean age 43.3 ± 15.4 years) received laparoscopic FNF with complete fundus mobilization (Group B). Clinical assessment was performed using a structured questionnaire and SF-36 quality of life (QoL) score. PD was assessed using a validated classification, and an overall outcome was also determined by asking the patient to score it. Results. At 24-month follow-up, 38 (88.3%) patients in Group A vs 39 (86.6%) in Group B reported to be completely satisfied with reflux relief and free of protonic pump inhibitors (PPIs), while 3 (6.9%) in Group A vs 2(4.4%) in Group B reported occasional PPI intake and 2(4.6%) in Group A vs 4(8.8%) in Group B needed regular PPI use. Persistent PD was observed in 8(18.6%) patients in Group A and in 2(4.4%) in Group B (P = .03). No significant differences were found in the QoL score and in the overall outcome perceived by the patients. Conclusion. FNF, with complete fundus mobilization, appears to be associated with a lower rate of postoperative persistent dysphagia.

Models of community-based primary care for epilepsy in low- and middle-income countries.

OBJECTIVE: To review systematically community-based primary care interventions for epilepsy in low- and middle-income countries to rationalize approaches and outcome measures in relation to epilepsy care in these countries. METHODS: A systematic search of PubMed, EMBASE, Global Index Medicus, CINAHL, and Web of Science was undertaken to identify trials and implementation of provision of antiseizure medications, adherence reinforcement, and/or health care provider or community education in community-based samples of epilepsy. Data on populations addressed, interventions, and outcomes were extracted from eligible articles. RESULTS: The 24 reports identified comprise mostly care programs addressing active convulsive epilepsy. Phenobarbital has been used most frequently, although other conventional antiseizure medications (ASMs) have also been used, but none of the newer. Tolerability rates in these studies are high, but overall attrition is considerable. Other approaches include updating primary health care providers, reinforcing treatment adherence in clinics, and raising community awareness. In these programs, the coverage of existing treatment gap in the community, epilepsy-related mortality, and comorbidity burden are only fleetingly addressed. None, however, explicitly describe sustainability plans. CONCLUSIONS: Cost-free provision, mostly of phenobarbital, has resulted in short-term seizure freedom in roughly half of the people with epilepsy in low- and middle-income countries. Future programs should include a range of ASMs. These should cover apart from seizure control and treatment adherence, primary health care provider education, community awareness, and referral protocols for specialist care. Programs should incorporate impact assessment at the local level. Sustainability in the long term as much as resilience and scalability should be addressed in future initiatives.

Evaluating the Surgeons' Perception of Difficulties of Two Techniques to Perform STARR for Obstructed Defecation Syndrome: A Multicenter Randomized Trial.

BACKGROUND: After initial enthusiasm in the use of a dedicated curved stapler (CCS-30 Contour Transtar) to perform stapled transanal rectal resection (STARR) for obstructed defecation syndrome (ODS), difficulties have emerged in this surgical technique. OBJECTIVE: First, to compare surgeons' perception of difficulties of STARR performed with only Transtar versus STARR performed with the combined use of linear staplers and Transtar to cure ODS associated with large internal prolapse and rectocele; second, to compare the postoperative incidence of the urge to defecate between the 2 STARR procedures. DESIGN AND SETTING: An Italian multicenter randomized trial involving 25 centers of colorectal surgery. PATIENTS: Patients with obstructed defecation syndrome and rectocele or rectal intussusception, treated between January and December 2012. INTERVENTIONS: Participants were randomly assigned to undergo STARR with a curved alone stapler (CAS group) or with the combined use of linear and curved staplers (LCS group). MAIN OUTCOME MEASURES: Primary end-points were the evaluation of surgeons' perception of difficulties score and the incidence of the "urge to defecate" at 3-month follow up. Secondary end-points included duration of hospital stay, rates of early and late complications, incidence of "urge to defecate" at 6 and 12 months, success of the procedures at 12 months of follow-up. RESULTS: Of 771 patients evaluated, 270 patients (35%) satisfied the criteria. Follow-up data were available for 254 patients: 128 patients (114 women) in the CAS group (mean age, 52.1; range, 39-70 years) and 126 (116 women) in LCS group (mean age, 50.7 years; range, 41-75 years). The mean surgeons' perception score, was 15.36 (SD, 3.93) in the CAS group and 12.26 (SD, 4.22) in the LCS group (P < .0001; 2-sample t test). At 3-month follow-up, urge to defecate was observed in 18 (14.6%) CAS group patients and in 13 (10.7%) LCS group patients (P = .34; Fisher's exact test). These values drastically decrease at 6 months until no urge to defecate in all patients at 12 months was observed. At 12-month follow-up, a successful outcome was achieved in 100 (78.1%) CAS group patients and in 105 (83.3%) LCS group patients (P = .34; Fisher's exact test). No significant differences between groups were observed in the hospital stay and rates of early or late complications occurring after STARR. CONCLUSIONS: STARR with Transtar associated with prior decomposition of prolapse, using linear staplers, seems to be less difficult than that without decomposition. Both procedures appear to be safe and effective in the treatment of obstructed defecation syndrome resulting in similar success rates and complications.

Transverse Perineal Support: A Novel Surgical Treatment for Perineal Descent in Patients With Obstructed Defecation Syndrome.

BACKGROUND: We hypothesized that pathological perineal descent may be responsible for the failure of operations for obstructed defecation syndrome and that correcting excessive perineal descent may improve the outcome in this group of patients. OBJECTIVE: The purpose of this study was to report the short-term preliminary results of a novel surgical procedure, transverse perineal support, for the correction of pathological perineal descent. DESIGN: This was a prospective, uncontrolled, open-label study. SETTINGS: The study was conducted in a hospital and a university center. PATIENTS: Among 25 patients observed with failure of previous surgery for obstructed defecation syndrome, 12 with pathological perineal descent underwent transverse perineal support, were followed-up at 6 months, and constituted the object of analysis. INTERVENTIONS: The surgical procedure was performed positioning a porcine dermal implant just above the perineum superficial fascia sutured to the periosteum membrane of ischiatic tuberosities at the insertion of the superficial transverse perineal muscle. MAIN OUTCOME MEASURES: The main outcome measures were obstructed defecation syndrome score and x-ray and magnetic resonance defecographic imaging evaluation of perineal descent and anorectal manometric parameters. RESULTS: The postoperative median obstructed defecation syndrome score was 7.0 (range, 3-8), showing a statistically significant difference if compared with the preoperative score of 13.5 (range, 9-18; p = 0.0005). The mean postoperative maximum intrarectal pressure was 69.4 ± 11.1 mm Hg, significantly higher than the preoperative pressure of 45.9 ± 12.8 mm Hg (p < 0.0001). At postoperative x-ray and magnetic resonance imaging defecography, the mean fixed and dynamic perineal descents were significantly lower than the preoperative descents (p = 0.02 for fixed perineal descent and p = 0.0004 for dynamic perineal descent). Of the 4 patients (33.3%) with preoperative pathological dynamic perineal descent, only 1 showed a persistent pathological dynamic perineal descent. No early or late complication was observed. LIMITATIONS: The study was limited by its small size and short follow-up time. CONCLUSIONS: Transverse perineal support appears to be a promising, safe, and effective procedure in the treatment of obstructed defecation syndrome associated with pathological perineal descent (see Video, Supplemental Digital Content 1, http://links.lww.com/DCR/A225).

Myoxinol (Hydrolyzed Hibiscus esculentus Extract) in the Cure of Chronic Anal Fissure: Early Clinical and Functional Outcomes.

Objective. This study was designed to evaluate the early results of the topical application of Hydrolyzed Hibiscus esculentus Extract 3% ointment (Myoxinol 3%), a novel local product with Botox-like activity, in the conservative treatment of chronic anal fissure (CAF). Methods. Among all patients with CAF observed during the study period, 31 subjects met the inclusion criteria and underwent medical therapy with Myoxinol 3% ointment every 12 hours for 6 weeks. Two patients were lost to follow-up. Clinical and manometric follow-up was carried out eight weeks after treatment. Results. At follow-up the success rate was 72.4% (21/29); median VAS score and mean anal resting pressure were significantly lower if compared with respective baseline data. The only one adverse effect of the topical application of Myoxinol 3% ointment was perianal itch, which was reported by 3,4% (1/29) of the patients available for the analysis. However, in this case this symptom did not cause interruption of the treatment. Conclusions. The topical application of Myoxinol 3% ointment in the cure of CAF shows encouraging early results. Further researches with a larger series and a longer follow-up are needed to confirm these data.

Role of tridimensional endoanal ultrasound (3D-EAUS) in the preoperative assessment of perianal sepsis.

PURPOSE: The aim of this study was to evaluate the accuracy of tridimensional endoanal ultrasound (3D-EAUS) in the diagnosis of perianal sepsis comparing the results with the surgical findings, considered as reference standard. METHODS: From January 2009 to January 2013, all the patients referred for the assessment and treatment of perianal sepsis with suspected anorectal origin were enrolled in the study. All patients gave informed written consent. Prior to surgery, all the patients underwent anamnestic evaluation, clinical examination, and unenhanced and H2O2-enhanced 3D-EAUS. Surgery was performed by a colorectal surgeon blinded to the 3D-EAUS results. RESULTS: A total of 212 patients with suspected perianal suppurations were assessed during the study period. In 12 patients, the H2O2-enhanced 3D-EAUS was not performed, and so, they were excluded from the study. Very good agreement between 3D-EAUS and examination under anesthesia (EUA) in the classification of primary fistula tracts (kappa = 0.93) and in the identification of fistula internal opening (kappa = 0.97) was found. There was a good concordance (kappa = 0.71) between 3D-EAUS and surgery in the detection of fistula secondary extensions. The overall sensitivity and specificity of 3D-EAUS in the diagnosis of perianal sepsis were 98.3 and 91.3% respectively. CONCLUSION: 3D-EAUS is a safe and reliable technique in the assessment of perianal sepsis. It may assist the surgeon in delineating the fistula tract anatomy and in determining the origin of sepsis, supporting the preoperative planning of definitive and appropriate surgical therapy.

Five-item score for obstructed defecation syndrome: study of validation.

The use of a dedicated score represents an essential tool for the clinical staging of obstructed defecation syndrome (ODS), for subsequent diagnostic and therapeutic options, and for the evaluation of the results. This study was designed to develop and validate a new, simple, and disease-specific scoring system for ODS. The questionnaire consists of 5 items: excessive straining, incomplete rectal evacuation, use of enemas and/or laxatives, vaginal-anal-perineal digitations, and abdominal discomfort and/or pain. Each item was graded from 0 to 5 with a score ranging from 0 (no symptoms) to 20 (very severe symptoms). A specific statistical analysis identifies the new score as a valuable and concise instrument, which demonstrates, overall, excellent concurrent validity, reproducibility, internal consistency, and discriminant validity for the diagnosis and grading of ODS. The use of this questionnaire may improve uniformity in clinical research and may allow a more precise evaluation of symptom severity and treatment effectiveness in ODS.

PPH-01 versus PPH-03 to perform STARR for the treatment of hemorrhoids associated with large internal rectal prolapse: a prospective multicenter randomized trial.

PURPOSE: This multicenter randomized study was designed to compare the clinical and functional results of stapled transanal rectal resection (STARR) performed with 2 staplers (PPH-01 vs. PPH-03) in the treatment of hemorrhoidal disease associated with a large internal rectal prolapse. METHODS: From a total of 937 patients, referred for hemorrhoidal disease in the 20 centers involved in the study, 425 (45.3%) with prolapsed hemorrhoids associated with a large internal rectal prolapse were randomized to undergo STARR with PPH-01 or PPH-03. Postoperative evaluation was made at 3, 6, and 12 months. RESULTS: The incidence of bleeding at the stapled line was significantly lower in the PPH-03 group than in the PPH-01 group (58/207 [28.0%] vs. 145/201 [72.1%]; P < .0001); the mean number of hemostatic stitches was significantly higher in the PPH-01 than in the PPH-03 group (3.2 ± 0.1 vs. 1.8 ± 0.8; P < .0001). The mean operative time was 25.1 ± 11.5 minutes in the PPH-03 group and 38.1 ± 15.7 minutes in the PPH-01 group (P < .0001). No major complications occurred in either of the groups. At 12-month follow-up, the success rate in the 2 groups was 94.5% in the PPH01 group and 94.2% in the PPH03 group. CONCLUSION: STARR performed for the treatment of hemorrhoidal disease associated with a large rectal prolapse is a safe and effective procedure. The use of the PPH-03 stapler instead of the PPH-01 guarantees a statistically significant reduction of intraoperative bleeding and a significant decrease of the operative time.

Improved clinical outcomes with a new contour-curved stapler in the surgical treatment of obstructed defecation syndrome: a mid-term randomized controlled trial.

BACKGROUND: Stapled transanal rectal resection has become the primary surgical procedure for surgical treatment of obstructed defecation syndrome caused by rectocele or rectal intussusception. The procedure is generally performed with 2 circular staplers. Recently, a dedicated contour-curved stapler was developed. OBJECTIVE: This study was designed to compare the effects of these stapler types on relief of symptoms. DESIGN AND SETTING: A randomized controlled trial was conducted at a regional referral center in Naples, Italy. PATIENTS: Patients with obstructed defecation syndrome and rectocele or rectal intussusception, treated from November 2005 through September 2007. INTERVENTIONS: Participants were randomly assigned to undergo stapled transanal rectal resection with 2 circular staplers or the contour-curved stapler. MAIN OUTCOME MEASURES: The primary end point was success rate at 24 months, defined by symptom improvement on an obstructed defecation syndrome scale. Secondary end points included success rate at 12 months, Agachan-Wexner constipation score, and rates of early and late complications at 12 and 24 months. RESULTS: Of 198 patients evaluated, 63 patients (31.8%) satisfied criteria. Follow-up data were available for 61 patients: 30 patients (28 women) in the circular stapler group (mean age, 53; range, 41-75 years) and 31 (29 women) in the contour-curved stapler group (mean age, 55; range, 38-69 years). At 24-month follow-up, success was achieved in 21 patients (70.0%) with the circular staplers and in 27 (87.0%) with the contour-curved stapler (P = .10). Symptom scores improved significantly in both groups from baseline to 12 months (P < .0001). Improvement was maintained in the contour-curved stapler group: mean score, 5.0 (SD, 1.6) at 12 months and 5.5 (1.5) at 24 months (P = .20). In the circular stapler group, symptom scores worsened from 4.5 (1.5) at 12 months to 9.0 (1.3) at 24 months (P < .0001). At 24 months, the groups differed significantly in symptom scores (P < .0001) and constipation scores (P = .03). No significant differences were seen in duration of postoperative hospital stay or rate of early or late complications. CONCLUSIONS: Stapled transanal rectal resection with either circular or contour-curved staplers can achieve relief of symptoms in patients with obstructed defecation syndrome. The contour-curved stapler appears to result in more stable clinical results over time.

[Prognostic and therapeutic value of D2 lymphadenectomy in the treatment of gastric cancer: experience of an Italian team]. / Valore prognostico e terapeutico della linfoadenectomia D2 nel trattamento chirurgico del cancro gastrico: considerazioni di un gruppo Italiano.

Recently, the incidence of gastric cancer and the related percentage of mortality have been decreasing world-wide, especially in the industrialised countries. Surgery has commonly been opted for as primary treatment of this disease. However, the optimal extent of surgical intervention is still debated. Japanese surgeons have been the pioneers of perigastric and celiac tripod lymphadenectomies in an attempt to improve long-term survival and the postoperative disease-free period. In recent years, D2 resection has been compared with D1 resection, which consists in excision of the stomach along with its locoregional nodes. From March 2002 to January 2007, 70 interventions for gastric cancer were performed at the VII Division of General Surgery of the Second University of Naples. All patients underwent excision of the lymph nodes from stations 1 to 6 (D1) combined with excision of stations 7 to 12 (D2). In 32 cases (45.7%) there were no metastatic lymph nodes (NO), in 28 patients (40%) 1 to 6 nodes proved metastatic (N1), and in 10 cases (14.3%) from 7 to 15 nodes were metastasised (N2). The incidence of metastatic lymph nodes was 54.3% and the prevalence 13.6%. Metastatic lymph nodes were found mostly in T3 (15/24) and T4 (14/20) stage tumours rather than in T1 (3/12) and T2 (6/14) neoplasms. Two patients (2.86%) died within 60 days of the intervention. The overall postoperative morbidity and mortality rates were 21.43% and 2.86%, respectively. D2 gastrectomies without pancreatic resections present distinct advantages in terms of long-term survival and are associated with postoperative morbidity and mortality rates which are similar to those obtained after D1 node resection.

Surgical therapy of goiter plunged in the mediastinum. Considerations regarding our experience with 165 patients.

Thyroid goiter is called plunged when, starting from the cervical region, it grows in the anterior-superior mediastinum to a depth of more than 2 cm. The highest frequency (48.44%) is evident in the age range from 60 to 70. From February 2002 to August 2005, performed 133 (80.6%) near total thyroidectomies, 26 total thyroidectomies (15.7%), 4 lobectomies (2.4%) and 2 (1.2%) totalisations of recurrences for plunged goiter in the 7th Division of General Surgery of the Second University of Naples. The goiters were classified using Lamke and Ferrante's topographical classification, which distinguishes between prevascular and retrovascular cervico-mediastinal goiters depending on the position assumed by the part plunged in the mediastinum in relation to the vascular layer of the epiaortic trunks. Retrovascular goiters are further subdivided into: pre-tracheal, laterovisceral and retrovisceral goiters depending on the relation to the trachea, oesophagus and epiaortic trunks. Among the patients who underwent near total thyroidectomy, there were 3 cases of permanent hypocalcaemia, 7 (7.2%) of temporary hypocalcaemia and only 1 (0.6%) monolateral temporary recurrent nerve lesion. Among those who underwent total thyroidectomy there was 1 case (0.6%) of temporary hypocalcaemia, and 1 (0.6%) of permanent hypocalcaemia. Postoperatively we also observed 3 cases (1.8%) of haemorrhage and 3 (1.8%) transfers to intensive care for respiratory insufficiency; 1 of these patients died 25 days after the operation. Ligature of the inferior and superior thyroid artery near the thyroid capsule protects the vascularisation of the parathyroid glands as well as the recurrent nerve and its division branches.