Small internal jugular veins with restricted outflow are associated with severe multiple sclerosis: a sonographer-blinded, case-control ultrasound study

Background: Recent evidence has indicated an association between chronic cerebrospinal venous insufficiency (CCSVI) and multiple sclerosis. Small internal jugular veins (IJVs) (with a cross-sectional area of less than 0.4 cm(2)) have been previously described as difficult to catheterize, and their presence may potentially affect cerebrospinal venous drainage. In this blinded extracranial color-Doppler study we had two principal aims: first, to assess prevalence of CCSVI among Serbian MS patients compared to healthy controls; and second, to assess prevalence of small IJVs (with a CSA LT = 0.4 cm(2)) among MS patients and controls. Methods: The sixty seven unrelated patients with clinical isolated syndrome (CIS), relapsing-remitting (RR), secondary progressive (SP) and primary progressive (PP) multiple sclerosis and 21 healthy controls were examined by high-resolution color-Doppler. Results: The ultrasonographic criteria of CCSVI (according to Zamboni) were positive in 11.9% of the patients and in none of the control subjects. The CCSVI-positive patients had significantly longer disease durations and were significantly more disabled (measured by their Expanded Disability Status Scale (EDSS) and Multiple Sclerosis Severity Score (MSSS) scores), but after adjustment for gender and disease duration, CCSVI was not an independent risk factor for multiple sclerosis severity. The small IJVs were found in 28.4% of the patients and 28.6% of the controls. The patients with small IJVs were associated with decreased venous outflow from the brain and presented with longer disease durations and significantly higher EDSS and MSSS scores compared to patients without small IJVs. A multivariate logistic regression analysis adjusted for gender and disease duration showed that small IJV is an independent factor associated with multiple sclerosis severity (EDSS GT = 6) (adjusted OR = 8.9, 95% CI: 1.8-45.6, p = 0.007). Among patients with small IJVs the 36.84% were also CCSVI positive. Conclusions: Both, CCSVI and small IJVs seem to influence or follow MS severity, but only small IJVs turned out to be an independent factor in this study. Thus, small IJVs with restricted outflow, which might be aspects of CCSVI different from the criteria originally described by Zamboni, emerge as a cofactor in the multifactorial pathophysiology of multiple sclerosis

Mortality from gastrointestinal congenital anomalies at 264 hospitals in 74 low-income, middle-income, and high-income countries: a multicentre, international, prospective cohort study

Summary Background Congenital anomalies are the fifth leading cause of mortality in children younger than 5 years globally. Many gastrointestinal congenital anomalies are fatal without timely access to neonatal surgical care, but few studies have been done on these conditions in low-income and middle-income countries (LMICs). We compared outcomes of the seven most common gastrointestinal congenital anomalies in low-income, middle-income, and high-income countries globally, and identified factors associated with mortality. Methods We did a multicentre, international prospective cohort study of patients younger than 16 years, presenting to hospital for the first time with oesophageal atresia, congenital diaphragmatic hernia, intestinal atresia, gastroschisis, exomphalos, anorectal malformation, and Hirschsprung’s disease. Recruitment was of consecutive patients for a minimum of 1 month between October, 2018, and April, 2019. We collected data on patient demographics, clinical status, interventions, and outcomes using the REDCap platform. Patients were followed up for 30 days after primary intervention, or 30 days after admission if they did not receive an intervention. The primary outcome was all-cause, in-hospital mortality for all conditions combined and each condition individually, stratified by country income status. We did a complete case analysis. Findings We included 3849 patients with 3975 study conditions (560 with oesophageal atresia, 448 with congenital diaphragmatic hernia, 681 with intestinal atresia, 453 with gastroschisis, 325 with exomphalos, 991 with anorectal malformation, and 517 with Hirschsprung’s disease) from 264 hospitals (89 in high-income countries, 166 in middleincome countries, and nine in low-income countries) in 74 countries. Of the 3849 patients, 2231 (58·0%) were male. Median gestational age at birth was 38 weeks (IQR 36–39) and median bodyweight at presentation was 2·8 kg (2·3–3·3). Mortality among all patients was 37 (39·8%) of 93 in low-income countries, 583 (20·4%) of 2860 in middle-income countries, and 50 (5·6%) of 896 in high-income countries (p<0·0001 between all country income groups). Gastroschisis had the greatest difference in mortality between country income strata (nine [90·0%] of ten in lowincome countries, 97 [31·9%] of 304 in middle-income countries, and two [1·4%] of 139 in high-income countries; p≤0·0001 between all country income groups). Factors significantly associated with higher mortality for all patients combined included country income status (low-income vs high-income countries, risk ratio 2·78 [95% CI 1·88–4·11], p<0·0001; middle-income vs high-income countries, 2·11 [1·59–2·79], p<0·0001), sepsis at presentation (1·20 [1·04–1·40], p=0·016), higher American Society of Anesthesiologists (ASA) score at primary intervention (ASA 4–5 vs ASA 1–2, 1·82 [1·40–2·35], p<0·0001; ASA 3 vs ASA 1–2, 1·58, [1·30–1·92], p<0·0001]), surgical safety checklist not used (1·39 [1·02–1·90], p=0·035), and ventilation or parenteral nutrition unavailable when needed (ventilation 1·96, [1·41–2·71], p=0·0001; parenteral nutrition 1·35, [1·05–1·74], p=0·018). Administration of parenteral nutrition (0·61, [0·47–0·79], p=0·0002) and use of a peripherally inserted central catheter (0·65 [0·50–0·86], p=0·0024) or percutaneous central line (0·69 [0·48–1·00], p=0·049) were associated with lower mortality. Interpretation Unacceptable differences in mortality exist for gastrointestinal congenital anomalies between lowincome, middle-income, and high-income countries. Improving access to quality neonatal surgical care in LMICs will be vital to achieve Sustainable Development Goal 3.2 of ending preventable deaths in neonates and children younger than 5 years by 2030

Review of laparoscopic management of mature cystic teratoma of ovaries in children

Ovarian cystic mature teratomas (OCMTs) are the most frequent ovarian tumors in childhood. This review aimed to determine the feasibility and safety of laparoscopic management of OCMT. Literature was searched for terms “mature,” “ovarian,” “teratomas,” and “laparoscopy.” Primary endpoints were age at surgery, laparoscopic and surgical technique, intraoperative complications, postoperative morbidity, and associated pathology. Literature search revealed 11 articles published between 1998 and 2014 that met the inclusion criteria. There were 105 (n = 95 unilateral; n = 10 bilateral) patients for this analysis, with mean age at surgery being 13 years. Four laparoscopic approaches were opted: gasless transumbilical laparoendoscopic single-site (LESS) surgery (n = 19), gasless multiport surgery (n = 24), single-incision laparoscopic surgery (SILS) (n = 3), and pneumoperitoneum multiport laparoscopy (n = 59). The 10 patients with bilateral OCMT underwent ovary-sparing surgery: LESS-assisted extracorporeal bilateral cystectomy in which tumors were punctured by a balloon catheter (n = 2), intracorporeal cystectomy for gasless multiport laparoscopy (n = 5) with use of endobags to prevent spillage, and transperitoneal multiport laparoscopy (n = 3). OCMT was associated with ipsilateral and unilateral ovarian torsion in five and bilateral ovarian torsion in one patient with bilateral OCMT. In four patients with unilateral OCMT, salpingo-oophorectomy was performed. Intraoperative complications were laceration of utero-ovarian ligament and bladder injury during a suprapubic port placement. The mean follow-up was 31.9 months. Patients with unilateral or bilateral OCMT can be offered ovarian-sparing surgery laparoscopically with one of the following techniques: LESS, SILS or multiport laparoscopy with pneumoperitoneal or gasless. Long-term follow-up of these techniques has shown no recurrence with low postoperative morbidity and low intraoperative complications

Laparoscopic management of Müllerian duct remnants in the paediatric age: Evidence and outcome analysis

Background: This study performed a literature analysis to determine outcomes of laparoscopic management in Müllerian duct remnants (MDRs). Patients and Methods: Literature was searched for terms 'Müllerian' 'duct' 'remnants' and 'laparoscopy'. Primary end points were age at surgery, laparoscopic technique, intraoperative complications and postoperative morbidity. Results: The search revealed 10 articles (2003–2014) and included 23 patients with mean age of 1.5 years (0.5–18) at surgery. All patients were 46XY, n = 1 normal male karyotype with two cell lines. Explorative laparoscopy was performed in n = 2 and surgical management in n = 21. The 5-port technique was used in n = 10, 3-port in n = 9 and robot-assisted laparoscopic approach in n = 1 (n = 1 technique not described). Complete MDRs removal in n = 9, complete dissection and MDRs neck ligation with endoscopic loops in n = 11 and n = 1 uterus and cervix were split in the midline. After MDRs removal, there were n = 2 bilateral orchidopexy, n = 3 unilateral orchidopexy, n = 1 Fowler–Stephens stage-I and n = 1 orchiectomy. Mean operative time was 193 min (120–334), and there were no intraoperative complications. Mean follow-up was 20.5 months (3–54) and morbidity included 1 prostatic diverticula. There were 13 associations with hypospadias, of which 3 had mixed gonads and 3 bilateral cryptorchidism. Other associations were unilateral cryptorchidism and incarcerated inguinal hernia n = 1, right renal agenesis and left hydronephrosis n = 1 and n = 2 with transverse testicular ectopy. Conclusion: This MDRs analysis suggests that the laparoscopic approach is an effective and safe method of treatment as no intraoperative complication has reported, and there is low morbidity in the long-term follow-up

Insulin resistance and multiple sclerosis: a pilot study in drug naive patients

7th Joint European-Committee-for-Treatment-and-Research-in-Multiple-Sclerosis (ECTRIMS)-Americas-Committee-for-Treatment-and-Research-in-Multiple-Sclerosis (ACTRIMS), Oct 25-28, 2017, Paris, Franc

Insulin resistance and multiple sclerosis: a pilot study in drug naive patients

7th Joint European-Committee-for-Treatment-and-Research-in-Multiple-Sclerosis (ECTRIMS)-Americas-Committee-for-Treatment-and-Research-in-Multiple-Sclerosis (ACTRIMS), Oct 25-28, 2017, Paris, Franc

Rare case of aneurymal bone cyst of cuboid bone in a 10 year old girl

Aneurysmal bone cysts (ABC) are rare and they represent  1- 1.4 % of all primary bone tumors. ABC of cuboid bone are extremely rare, especially in children a very  few cases have been reported in literature since 1967.  We  present a case of pathological fracture of cuboid bone due to aneurysmal cyst in a 10 year old girl. Surgery was performed, which included  open biopsy with aspiration and intralesional curettage . Bone defect was then filled in by fully synthetic cancellous bone graft substitute, consisting of pure β-tricalcium phosphate. Aneurysmal cyst of cuboid bone with pathological fracture could be successfuly treated with pure β-tricalcium phosphate as a bone graft substitute. This procedure is safe with excellent outcome.Follow up to nearly four years did not show any reccurence

Matrix metalloproteinase-9-1562 C/T gene polymorphism in Serbian patients with multiple sclerosis

Matrix metalloproteinase-9 (MMP-9) is suggested to play a role in MS by mediating T cell migration across subendothelial basement membrane and by contribution to myelin breakdown. We studied the association of MMP-9 -1562 C/T gene polymorphisms with MS susceptibility and severity in 187 patients from Serbia. The significant decrease in T allele carriership (p=0.01), was found in female NIS patients, In addition, a trend toward lower MSSS in T allele carriers was noticed (CC, mean 5.7 +/- 2.5 vs. CT+TT, mean 4.9 +/- 2.5). Further studies in different populations are needed to resolve the potential influence of MMP-9 gene polymorphism on MS. (C) 2007 Elsevier B.V. All rights reserved

Association of two polymorphisms in IL-1 beta and IL-1RA genes with susceptibility for multiple sclerosis

15th Meeting of the European-Neurological-Society, Jun 18-22, 2005, Vienna, Austri

Association of two polymorphisms in IL-1 beta and IL-1RA genes with response to treatment with Betaferon

15th Meeting of the European-Neurological-Society, Jun 18-22, 2005, Vienna, Austri