Addendum to the consensus opinion from the International Deep Endometriosis Analysis (IDEA) group: sonographic evaluation of the parametrium.

Preoperative sonographic staging in patients with suspected parametrial endometriosis is essential to plan the surgical intervention and to anticipate the need for a multidisciplinary approach, and hence optimize surgical outcome. The results of a recent metanalysis suggest that defining more accurately the ultrasonographic criteria of parametrial involvement in endometriosis is needed. The aim of this addendum to the IDEA-consensus is to highlight the sonographic characteristics of the parametrium and identify ultrasound techniques to diagnose deep endometriosis in this area. This article is protected by copyright. All rights reserved.

High-Dose Levetiracetam for Neonatal Seizures: A Retrospective Review.

BACKGROUND: Neonatal seizures are frequently encountered in the neonatal intensive care unit and may be associated with serious long-term neurological sequelae. Response to treatment continues to be modest, and treatment guidelines remain unclear. The use of levetiracetam has been on the rise in the past several years due to its favorable safety profile in the face of limited data on its efficacy and optimal dosing regimens. Unlike the older age groups, the benefit of escalating to high-dose levetiracetam of 80-100 mg/kg/day in neonates not responding to the standard used dosing regimen (40-60 mg/kg/day) is not studied. We sought to investigate the safety and efficacy of levetiracetam escalation to high dose regimens for neonatal seizures. METHODS: A retrospective chart review over a 7-year period was conducted at the American University of Beirut to identify neonates with electrographically proven seizures treated with levetiracetam. Data was collected on electroclinical seizure characteristics, underlying etiology, seizure control, other anti-seizure medications, and adverse effects. RESULTS: Electronic chart review revealed a total of 15 neonates with electrographically confirmed seizures treated with levetiracetam, with escalation to high doses in seven. As a first line drug, levetiracetam monotherapy terminated seizures in six out 10 neonates, two of whom had complete seizure cessation only after escalation to high doses of 80 or 100 mg/kg/day. When used in combination with other anti-seizure medications, four out of five neonates achieved complete seizure cessation upon escalation to high doses of levetiracetam. No adverse effects were noted. CONCLUSIONS: In neonates not responding to the standard used levetiracetam doses, incremental increases to 80-100 mg/kg/day may be considered. Prospective studies are needed to confirm the promising role of such high dosing regimens, and to better elucidate the role of levetiracetam in neonatal seizures.

Outcome of Nonketotic Hyperglycinemia in Lebanon: 14-Year Retrospective Review.

Nonketotic hyperglycinemia is a rare inborn error of glycine metabolism characterized by a severe metabolic encephalopathy with drug-resistant seizures. Here, we report the outcome of nonketotic hyperglycinemia in a cohort of patients diagnosed and followed-up at a tertiary care reference center in Lebanon, between 2000 and 2014.Eight out of 12 patients with nonketotic hyperglycinemia were retrospectively reviewed. The remainders were excluded for incomplete data. The majority of cases presented with seizures and hypsarrhythmia or burst suppression patterns. Half of the patients died. Survival varied between 7 days and 18 years. Seizures remained unresponsive with poor outcome, despite standard supportive care and antiepileptic therapy; however, two patients were responsive to ketogenic diet and one of them became seizure-free.Scarce data on the outcome of nonketotic hyperglycinemia patients from the Middle East and North Africa region are available. The ketogenic diet, in combination with standard therapies, appears to be effective in controlling the seizures in this devastating disorder. Larger multicenter studies are still needed to establish the role of the ketogenic diet in nonketotic hyperglycinemia.

Learning curve for ultrasonographic diagnosis of deep infiltrating endometriosis using structured offline training program.

OBJECTIVE: To assess the learning curves of trainees during a structured offline/hands-on training program for the ultrasonographic diagnosis of deep infiltrating endometriosis (DIE). METHODS: Four trainees (all Ob/Gyn postgraduates with at least 5 years' experience in ultrasonography in obstetrics and gynecology, but with no experience of sonographic examination of DIE) participated in the study. They underwent a 2-week training program with a single trainer. Day 1 was devoted to theoretical issues and guided offline analysis of 10 three-dimensional ultrasound volumes. During the following days, four sessions of real-time sonographic examinations were performed in a DIE referral center ultrasound unit. In between these sessions, the trainees analyzed four datasets offline, each containing 25 volumes. At the end of each set, misinterpreted volumes were reassessed with the trainer. Presence or absence of DIE at surgery was considered the gold standard. The trainees' learning process was evaluated by learning-curve cumulative summation (LC-CUSUM) and the deviations of the trainees' level of performance at the control stage was assessed by CUSUM (standard CUSUM), for different locations of DIE. RESULTS: The trainees reached competence after an average of 17 (range, 14-21) evaluations for bladder, 40 (range, 30-60) for rectosigmoid, 25 (range, 14-34) for forniceal, 44 (range, 25-66) for uterosacral ligament (USL) and 21 (range, 14-43) for rectovaginal septum (RVS) locations of DIE, and then kept the process under control, with error levels of less than 4.5% until the end of the test. The overall accuracy for each trainee in diagnosis of DIE at the different locations ranged from 0.91 to 0.98 for bladder DIE, from 0.80 to 0.94 for rectosigmoid DIE, from 0.90 to 0.94 for forniceal DIE, from 0.79 to 0.82 for USL DIE and from 0.89 to 0.98 for RVS DIE. CONCLUSIONS: The suggested 2-week training program, based on a mixture of offline and live scanning sessions, is feasible and apparently provides effective training for the ultrasonographic diagnosis of DIE. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Pulmonary hypertension is associated with higher mortality in cystic fibrosis patients awaiting lung transplantation.

AIM: Pulmonary hypertension (PH) is frequently found in patients with advanced parenchymal lung diseases. In advanced stages, cystic fibrosis (CF) patients can develop PH and eventually cor pulmonale. Little is known about the prevalence of PH in CF patients and its impact on outcome. METHODS: We retrospectively studied a large cohort of CF patients evaluated for lung transplantation between 1995 and 2010. All the patients underwent right heart catheterization as part of the evaluation. We included 179 unique consecutive adult CF patients. Age was 24±9 years and 45.8% were women. RESULTS: Eighty-seven patients were transplanted (48.6%) and 65 died (36.3%) while waiting for LT. By right heart catheterization, 38.5% of the patients had PH (mean ≥25 mm Hg). PaCO(2) (P=0.045) and forced vital capacity (P=0.023) were independent predictors of PH in CF patients. The median survival (free of lung transplantation) was 13.4 months. After adjusting for several covariates, the presence of PH significantly increased mortality (hazard ratio, HR) (P<0.001). Pulmonary vascular resistance was associated with mortality (P=0.03). When both PH and PVR were included in the model, only PH predicted mortality. CONCLUSION: Pulmonary hypertension of mild degree is frequently found in CF patients with advanced lung disease and its presence significantly worsens survival.

Pulmonary hypertension is associated with higher mortality in cystic fibrosis patients awaiting lung transplantation.

AIM:Pulmonary hypertension (PH) is frequently found in patients with advanced parenchymal lung diseases. In advanced stages, cystic fibrosis (CF) patients can develop PH and eventually cor pulmonale. Little is known about the prevalence of PH in CF patients and its impact on outcome. METHODS: We retrospectively studied a large cohort of CF patients evaluated for lung transplantation between 1995 and 2010. All the patients underwent right heart catheterization as part of the evaluation. We included 179 unique consecutive adult CF patients. Age was 24±9 years and 45.8% were women. RESULTS:Eighty-seven patients were transplanted (48.6%) and 65 died (36.3%) while waiting for LT. By right heart catheterization, 38.5% of the patients had PH (mean ≥25 mm Hg). PaCO2 (P=0.045) and forced vital capacity (P=0.023) were independent predictors of PH in CF patients. The median survival (free of lung transplantation) was 13.4 months. After adjusting for several covariates, the presence of PH significantly increased mortality (hazard ratio, HR) (P<0.001). Pulmonary vascular resistance was associated with mortality (P=0.03). When both PH and PVR were included in the model, only PH predicted mortality. CONCLUSION: Pulmonary hypertension of mild degree is frequently found in CF patients with advanced lung disease and its presence significantly worsens survival.

Management and prognosis of endometrioid borderline tumors of the ovary.

BACKGROUND: The Endometrioid Borderline ovarian tumor (EBOT) is the third most common histological subtype of borderline ovarian tumors. Very little is known about the prognosis and management of this entity. This paper consists of a review of the literature and an analysis of clinical series. STUDY DESIGN: A review of the literature on this topic was conducted identifying series reporting consecutive cases of EBOT using 2 search engines (MEDLINE and Pubmed). Personal data on this topic have been included and concern a series of patients treated between 1985 and 2009 for EBOT. These cases included in this series had complete data concerning patient management and follow-up > 12 months. RESULTS: 16 patients were studied: 7 had been treated conservatively and 9 radically. All 16/16 patients had stage I disease at the initial diagnosis but one patient had also developed synchronous endometrioid adenocarcinoma of the uterine corpus. After a median time of 24 months (range, 12-132) post treatment, one (1/16) patient had developed two recurrences. She remains disease-free 42 months after the end of treatment of the last recurrence. These data were compared to the results of 4 series previously reported in the literature. In fact, the present series reports on the first recurrence in EBOT (which was an invasive lesion). CONCLUSION: Endometrioid borderline ovarian tumors carry a good prognosis. Most EBOT tumors are stage I, therefore surgical staging is not necessary in most of the cases. However, uterine curettage is required in cases of uterine preservation.

Lung transplantation for cystic fibrosis after thoracic surgical procedures.

During their life, cystic fibrosis (CF) patients may require thoracic surgical procedures for a number of reasons before undergoing lung transplantation. In the past, this has been considered to be a contraindication to lung transplantation. However, a meticulous surgical technique and careful intraoperative management allows one to perform the transplantation safely. Herein we have reported our experience with CF patients undergoing lung transplantation after previous surgical treatment for pneumothorax or bronchiectasis.

Clostridium difficile-related pancolitis in lung-transplanted patients with cystic fibrosis.

C. difficile (C. d.) is the main cause of antibiotic-associated diarrhea and colitis. It is shown in literature a high asymptomatic carriage rate of C. d. in patients with cystic fibrosis (CF), though C. d.-related colitis is an uncommon complication in these patients, despite the use of multiple high-dose antibiotic regimes and the frequency of hospital admissions. Lung transplantation with the associated immunosuppression and aggressive antibiotic therapy may increase the risk of the clinical manifestation of C. d. In this paper, we describe three cases of severe C. d. colitis in patients with CF following lung transplantation and illustrate our experience in the diagnosis and management of these patients.

Nerve root enhancement on spinal MRI in pediatric Guillain-Barré syndrome.

Guillain-Barré syndrome diagnosis is based on clinical presentation and supportive diagnostic testing. In its early stage, no single, reliable diagnostic test is available. However, a finding of nerve root enhancement on spinal magnetic resonance imaging may be useful. We evaluated the frequency of nerve root enhancement on spinal magnetic resonance imaging in children with Guillain-Barré syndrome. At a single tertiary pediatric center, we conducted a retrospective chart review of children with Guillain-Barré syndrome who had complete spinal or lumbosacral spinal magnetic resonance imaging with gadolinium administration from January 2002-January 2009. Twenty-four consecutive patients were identified. Spinal nerve root enhancement with gadolinium was present in 92% (22/24) of children with Guillain-Barré syndrome on initial spinal magnetic resonance imaging (95% confidence interval, 0.745-0.978). This finding increased to 100% of patients, after two patients underwent repeat spinal magnetic resonance imaging that did reveal nerve root enhancement. Patterns of enhancement were variable, but involved the thoracolumbar nerve roots in all patients. Enhancement of nerve roots with gadolinium on initial spinal magnetic resonance imaging was frequently present in these children with Guillain-Barré syndrome. Spinal magnetic resonance imaging is a sensitive diagnostic test and should be considered an additional diagnostic tool in select cases.

Treatment of complex airway lesions after lung transplantation with self-expandable nitinol stents: early experience.

Airway complications (AC) are considered a serious cause of morbidity after lung transplantation (LT). Mechanical dilatation, laser vaporization, and silicone stent placement usually solve it. However, the use of self-expandable metallic stents (SENS) may be indicated in selected cases. Ten lung transplant recipients with AC were treated with SENS. Six patients underwent LT for cystic fibrosis, 2 for idiopathic pulmonary fibrosis, 1 for bronchiectasis, and 1 for emphysema. All patients received at least 1 treatment attempt with dilatation and silicone stent placement. The indications for SENS placement were the presence of a tortuous airway axis with stenosis and malacia of the right main bronchus in 5 patients; a long stenosis of the main and intermediate right bronchus involving the upper lobe orifice in 3 patients; or malacia that could not be stabilized with silicone stents in 3 cases. In 1 patient the procedure was bilateral. Functional improvement was immediate with a mean forced expiratory volume at 1 second (FEV(1)) gain of 35%. No stent dislocation was observed. Symptoms did not occur again in 5 patients with previous recurrent episodes of pneumonia. One stenosis, which was due to the ingrowth of granulation tissue occurred at 6 months after the procedure, was successfully treated with mechanical dilatation and laser vaporization. The deployment of SENS in a selected group of patients with AC after LT was easy, safe, and effective.

Extracorporeal circulatory support for lung transplantation: institutional experience.

Lung transplantation (LT) represents the only available therapy for selected patients affected by end-stage pulmonary disease. Cardiopulmonary bypass (CPBP) is used, when required, during single and sequential double lung transplantation; however, it increases the risk of bleeding, early graft dysfunction, failure, and other potential side effects. We report our experience with 145 patients who underwent lung transplantations, among whom 34 required intraoperative CPBP. The indications for LT among these 34 patients were cystic fibrosis (n = 22), chronic obstructive pulmonary disease (n = 3), bronchiectasis (n = 2), primary pulmonary hypertension (n = 1), fibrosis (n = 2), pulmonary microlithiasis (n = 1), and retransplantation for obliterative bronchilitis (n = 3). CPBP was planned in 12 cases (group I) and unplanned in 22 (group II). The main reason for planning CPBP was primary and secondary pulmonary hypertension (mean pulmonary artery pressure >or=25 mm Hg). Acute right ventricular failure, hemodynamic instability, arterial desaturation, and increased pulmonary artery pressure were mandatory for unplanned CPBP. Among the 34 CPBP patients, the 30-day mortality rate was 35% (12/34) including 9 (70%) in group II (unplanned CPBP). The leading cause of death was multiorgan failure. The 1-year survival rates were 67% and 36%, and the 3-year survival rates were 47% and 18% for groups I and II, respectively. In conclusion, even if it represents a useful tool in the management of critical events, the use of unscheduled CPBP during LT procedures is associated with an increased postoperative morbidity and mortality.

Pelvic lymphadenectomy in endometrial cancer: our current experience.

OBJECTIVE: Lymph node involvement is the single most important factor in the prognosis of endometrial cancer, because it is predictive of locoregional and distant metastases. The purpose of our study was to determine whether lymphadenectomy is useful in the surgical staging of endometrial cancer and if it may help establish a more accurate prognosis and reduce the need for postoperative therapy in patients without surgical complications. STUDY DESIGN: We conducted a retrospective study on 55 patients with diagnosis of endometrial cancer. RESULTS: Surgical staging of patients undergoing pelvic lymphadenectomy (47/55) showed that 59.6% of cases (n = 28) had Stage I cancer (IA in 4, IB in 16, IC in 8), 17.02% (n = 8) Stage II (IIA in 3, IIB in 5), 21.2% (n = 10) Stage III (IIIB in 5, IIIC in 5), and 2.1% (n = 1) Stage IVA. In the remaining eight patients with a very high anesthesiologic risk (ASA 4), surgical staging was incomplete because they underwent only node palpation. CONCLUSION: In conclusion, as we wait for the sentinel lymph node technique to demonstrate satisfactory results and be standardized also for endometrial cancer, we believe that surgical lymph node dissection plays a crucial role in debulking this type of cancer. When performed by a good surgical oncology team, it does not entail a significantly increased operative risk.

A perspective study on correlation between HPV DNA and lymph nodes in surgically treated cervical carcinoma patients. Preliminary data.

UNLABELLED: The purpose of this study was to analyze the presence of HPV DNA in lymph nodes in patients with cervical cancer. STUDY DESIGN: A prespective study was performed on a total of 18 patients with cervical cancer in FIGO Stage I-II. The surgical procedure consisted of systematic pelvic lymphadenectomy with removal of the common/external/internal (obturator) iliac lymph node chains, followed by radical hysterectomy depending on the clinical stage, or by Piver's type II radical laparohysterectomy for Stage IA2 carcinoma and Piver's type-III laparohysterectomy for Stage IB or Stage II carcinoma. After removal by a technique not yet described in the literature, the lymph nodes were processed directly in the operating room. HPV DNA testing was done using a cytobrush device. At the end of this operation, the lymph nodes were sent to the hospital's pathologist for metastasis detection. RESULTS: The correlation between a positive HPV DNA test in the cervix and lymph node metastasis was non significant (p < 0.63). By contrast, the correlation between a positive HPV DNA test in the lymph nodes and lymph node metastasis was highly significant (p < 0.005), as was the correlation between positive HPV DNA tests in the cervix and lymph nodes (p < 0.005). Finally, the correlation between disease stage and positive HPV DNA testing in the lymph nodes was also significant (p < 0.05). CONCLUSIONS: In conclusion, the technique that we used for HPV DNA extraction appears safe and reproducible. The results are comparable with, if not better, than those obtained with other techniques reported in the literature. The presence of HPV DNA in the lymph nodes is probably an early indicator of metastasis and as such it could be used as a predictor of relapse. Normally untreated patients who have this marker could then receive adjuvant therapy.

Incidence of port-site metastasis after laparoscopic management of borderline ovarian tumors: a series of 22 patients.

PURPOSE: The aim of this work was to evaluate the incidence of port-site metastasis in patients undergoing laparoscopy for borderline ovarian carcinoma (BOT). METHODS: Twenty-two patients who underwent laparoscopy from 2004 to 2008 for BOT were evaluated retrospectively. RESULTS: In 15 patients an ultraconservative procedure with enucleation of the annexal neoplasia was carried out, while in five (23%) unilateral salpingo-oophorectomy was performed and in two cases (9%) bilateral salpingo-oophorectomy was done. CONCLUSION: The literature data report few cases of port-site metastasis in BOT patients. Residual cutaneous metastases have been reported to occur within 12 months from the first surgery, generally in association with serous histology. In our analysis, we found 17 out of 22 cases of serous BOT, three mucinous and two endometriod. In no case was cutaneous metastasis revealed after an average of 30 months of follow-up.

Drugs of choice for sedation and analgesia in the neonatal ICU.

Painful procedures in the neonatal ICU are common, undertreated, and lead to adverse consequences. The drugs most commonly used to treat neonatal pain include the opiates, benzodiazepines, barbiturates, ketamine, propofol, acetaminophen, and local and topical anesthetics. This article discusses the indications for and advantages and disadvantages of the commonly used analgesic drugs. Guidance and references for drugs and dosing for specific neonatal procedures are provided.

Drugs of choice for sedation and analgesia in the neonatal ICU.

Painful procedures in the neonatal ICU are common, undertreated, and lead to adverse consequences. The drugs most commonly used to treat neonatal pain include the opiates, benzodiazepines, barbiturates, ketamine, propofol, acetaminophen, and local and topical anesthetics. This article discusses the indications for and advantages and disadvantages of the commonly used analgesic drugs. Guidance and references for drugs and dosing for specific neonatal procedures are provided.

Behaviour of ovarian tumors of low malignant potential treated with conservative surgery.

OBJECTIVE: Fertility-sparing surgery has been proposed for the treatment of borderline ovarian tumors. The aim of this study was to evaluate the outcome of patients submitted to cystectomy (CYS) compared with patients treated by unilateral salpingo-oophorectomy (USO) or bilateral salpingo-oophorectomy with/without total hysterectomy (radical surgery, RS). METHODS: We reviewed retrospectively the data of patients treated in 3 institutions for borderline ovarian tumors. One hundred and sixty-eight patients underwent laparoscopic or laparotomic surgical treatment from 1985 to 2006. Tumor recurrence rate, disease-free survival and site of recurrences were evaluated. Specific prognostic factors, such as stage, histology, micropapillary subtype, exophytic tumor growth, intraoperative spillage, endosalpingiosis, staging procedures, and route of surgery were analysed. RESULTS: Thirty-five patients underwent cystectomy, 50 unilateral salpingo-oopohorectomy, and 83 radical surgery. Twelve patients in the CYS group (34.3%), 10 in the USO group (20.0%), and 5 (6.0%) in RS group relapsed. Five-year progression-free survival (PFS) was 59.6%, 78.4%, and 93.5% in CYS, USO and RS groups, respectively. None of the relapsed patients died of disease. CONCLUSIONS: Cystectomy is an effective surgical strategy for patients with borderline ovarian tumor. The higher risk of local relapses is not associated with a reduction in the overall survival. The procedure should be offered to young patients with bilateral tumors and to very young ones, considering the higher risk of local relapse.

Lung transplantation for cystic fibrosis in Italy.

Lung transplantation (LT) is the only effective form of therapy for cystic fibrosis (CF) associated with end-stage pulmonary failure. In Italy, the management of CF is regulated by national law, which has instituted regional centers for care and follow-up of all CF patients. LT has been performed since 1992 in only nine LT certified centers. The structured national organization has led to a unified database for LT for CF. As of December 2006, 197 bilateral LT (96 male and 94 female patients; 7 retransplants) have been performed. Of these, four had also liver or heart and liver transplantation, and three are long-term survivors. Overall median survival is 7 years. Mean age at transplantation is 26.5 years, and the mortality on the waiting list is 33.6%. Patients listed for transplant either received a suitable donor within a mean of 10 months or died within a mean of 5.5 months. The most frequent cause of death is bronchiolitis obliterans syndrome (BOS). Our nationwide database indicates the excellent results obtained by LT in FC. Still, mortality on the waiting list remains a challenge and long-term outcome is limited by BOS.