Long-term survival after self-expanding metallic stent or stoma decompression as bridge to surgery in acute malignant large bowel obstruction.

AIM: Self-expanding metallic stents (SEMS) as bridge to surgery have been questioned due to the fear of perforation and tumour spread. This study aimed to compare SEMS and stoma as bridge to surgery in acute malignant large bowel obstruction in the Swedish population. METHOD: Medical records of patients identified via the Swedish Colorectal Cancer Register 2007-2009 were collected and scrutinized. The inclusion criterion was decompression intended as bridge to surgery due to acute malignant large bowel obstruction. Patients who underwent decompression for other causes or had bowel perforation were excluded. Primary endpoints were 5-year overall survival and 3-year disease-free survival. Secondary endpoints were 30-day morbidity and mortality rates. RESULTS: A total of 196 patients fulfilled the inclusion criterion (SEMS, 71, and stoma, 125 patients). There was no significant difference in sex, age, ASA score, TNM stage and adjuvant chemotherapy between the SEMS and stoma groups. No patient was treated with biological agents. Five-year overall survival was comparable in SEMS, 56 per cent (40 patients), and stoma groups, 48 per cent (60 patients), P = 0.260. Likewise, 3-year disease-free survival did not differ statistically significant, SEMS 73 per cent (43 of 59 patients), stoma 65 per cent (62 of 95 patients), P = 0.32. In the SEMS group, 1.4 per cent (one patient) did not fulfil resection surgery compared to 8.8 per cent (11 patients) in the stoma group (P = 0.040). Postoperative complication and 30-day postoperative mortality rates did not differ, whereas the duration of hospital stay and proportion of permanent stoma were lower in the SEMS group. CONCLUSION: This nationwide registry-based study showed that long-term survival in patients with either SEMS or stoma as bridge to surgery in acute malignant large bowel obstruction were comparable. SEMS were associated with a lower rate of permanent stoma, higher rate of resection surgery and shorter duration of hospital stay.

De novo and inherited mutations in the X-linked gene CLCN4 are associated with syndromic intellectual disability and behavior and seizure disorders in males and females.

Variants in CLCN4, which encodes the chloride/hydrogen ion exchanger CIC-4 prominently expressed in brain, were recently described to cause X-linked intellectual disability and epilepsy. We present detailed phenotypic information on 52 individuals from 16 families with CLCN4-related disorder: 5 affected females and 2 affected males with a de novo variant in CLCN4 (6 individuals previously unreported) and 27 affected males, 3 affected females and 15 asymptomatic female carriers from 9 families with inherited CLCN4 variants (4 families previously unreported). Intellectual disability ranged from borderline to profound. Behavioral and psychiatric disorders were common in both child- and adulthood, and included autistic features, mood disorders, obsessive-compulsive behaviors and hetero- and autoaggression. Epilepsy was common, with severity ranging from epileptic encephalopathy to well-controlled seizures. Several affected individuals showed white matter changes on cerebral neuroimaging and progressive neurological symptoms, including movement disorders and spasticity. Heterozygous females can be as severely affected as males. The variability of symptoms in females is not correlated with the X inactivation pattern studied in their blood. The mutation spectrum includes frameshift, missense and splice site variants and one single-exon deletion. All missense variants were predicted to affect CLCN4's function based on in silico tools and either segregated with the phenotype in the family or were de novo. Pathogenicity of all previously unreported missense variants was further supported by electrophysiological studies in Xenopus laevis oocytes. We compare CLCN4-related disorder with conditions related to dysfunction of other members of the CLC family.

New insights into Brunner syndrome and potential for targeted therapy.

We report two families with Brunner syndrome living in one state of Australia. The first family had a predicted protein-truncating variant of monoamine oxidase A (MAOA) (p.S251KfsX2). Affected males had mild intellectual disability (ID), obsessive behaviour, limited friendships and were introverted and placid during clinical interview. The family disclosed episodic explosive aggression after a diagnosis was made. The second family had a missense variant in MAOA (p.R45W). Affected males had borderline-mild ID, attention deficit disorder and limited friendships. One had a history of explosive aggression in childhood and episodic symptoms of flushing, headaches and diarrhoea. Their carrier mother had normal intelligence but similar episodic symptoms. Characteristic biochemical abnormalities included high serum serotonin and urinary metanephrines and low urinary 5-hydroxyindoleacetic acid (5-HIAA) and vanillylmandelic acid (VMA). Symptomatic individuals in the second family had particularly high serotonin levels, and treatment with a serotonin reuptake inhibitor and dietary modification resulted in reversal of biochemical abnormalities, reduction of 'serotonergic' symptoms and behavioural improvement. Brunner syndrome should be considered as a cause of mild ID with paroxysmal behavioural symptoms. It can be screened for with serum/urine metanephrine and serotonin measurement. Cautious treatment with a serotonin reuptake inhibitor, dietary modifications and avoidance of medications contraindicated in patients on monoamine oxidase inhibitors can improve symptoms.

Comparison between distally based peroneus brevis and sural flaps for reconstruction of foot, ankle and distal lower leg: an analysis of donor-site morbidity and clinical outcome.

BACKGROUND: Skin defects of the foot, ankle and distal lower leg often require coverage by local or distant flaps. We aimed to compare functional outcome and donor-site morbidity following transfer of distally based delayed sural (DSFs) or peroneus brevis flaps (PBFs). METHODS: Between 2003 and 2006, 52 patients (24 DSFs and 28 PBFs) were included. For increased reliability, all extended sural flaps were delayed for 3-15 days. At the end of the follow-up period (minimum 12 months), patients were asked to fill out a modified foot and ankle score (Foot and Ankle Outcome Score (FAOS)) questionnaire. In addition, a chart review and a physical examination were performed. RESULTS: Total hospital stay and total number of operations were significantly lower in the PBF group. Minor flap necrosis (<10%) was observed in 21% of the DSF and 7% of the PBF group, and partial (>10%) or total flap loss occurred in one and three patients from the DSF group, respectively. Patient satisfaction, FAOS results and range of motion were comparable in both groups. Defect aetiology and patient age did not influence surgical outcome. CONCLUSION: Donor-site morbidity and functional outcome after DSF and PBF are comparable. A higher rate of complications was observed in the DSF group. Based on our findings, the PBF is recommended as first-line procedure for reconstruction of small- to medium-sized defects at the distal tibia, fibula, ankle and heel. The sural flap might be chosen for extended skin defects especially when a larger arc of rotation is required.

The use of the artificial dermis (Integra) in combination with vacuum assisted closure for reconstruction of an extensive burn scar--a case report.

The artificial dermis Integra (Ethicon, Johnson & Johnson Medical, Norderstedt, Germany) is widely used in the treatment of excessive burn injuries. It is also used in reconstructive surgery when large soft-tissue defects could not be covered with local or free flaps. In this article a 25-year old patient who presented with an early childhood burn of the trunk and lower extremity was treated with Integra in combination with the vacuum assisted closure (V.A.C., KCI, Texas, U.S.A.) and split thickness skin grafting. The combination of the artificial dermal substitute with negative pressure therapy has lead to a complete healing of Integra and the skin graft. During the whole treatment sterile wound conditions were present and time-consuming dressing changes could be prevented. Hospital stay was shortened because the patient could be treated as an outpatient with an ambulant vacuum assisted closure device.

[Economic aspects of surgical wound therapies]. / Okonomische Aspekte in der chirurgischen Wundbehandlung.

INTRODUCTION: Health care insurers in Germany pay 5 billion Euros annually in materials alone for pressure ulcers, diabetic foot ulcers, and ulcus cruris. With such figures it is necessary to consider, economic aspects of treatment. METHODS: Due to the lack of evidence-based data on wound treatment costs, we investigated available studies for an effect on treatment costs when standard moist wound therapy was compared with alternative methods. Suited medical parameters are calculated and compared. Daily costs and length of treatment are correlated and compared. RESULTS: Published data show that alternative wound therapies may lead to an earlier wound closure, fewer complications, and reduction in hospital admissions and length of stay. Despite higher daily costs, some alternative wound therapies turn out to be more cost effective, when all economical factors are considered. In this respect a move towards alternative wound therapies could possibly lead to major savings. DISCUSSION: At present there is insufficient evidence to prove the efficacy of various treatment modalities for chronic wounds. This is due to numerous factors such as comorbidities and frequent multimorbidity. Nevertheless critical evaluation of one innovative treatment alone already uncovered an enormous potential for savings in a wider economic context, despite the comparatively higher cost of a single treatment. It is of utmost importance that conservative wound care become firmly embedded in surgical concepts.

[Vacuum assisted closure (V.A.C.) therapy is an essential tool for treatment of complex defect injuries of the upper extremity]. / Die Vakuumtherapie ist ein essenzielles Werkzeug in der Behandlung von komplexen Defektverletzungen der oberen Extremität.

INTRODUCTION: Complex injuries of the upper extremity remain a therapeutic challenge. The prognosis of the damaged extremity is often limited by soft tissue defects. Vacuum Assisted Closure (V.A.C.) therapy facilitates temporary coverage of soft tissue defects prior to surgical reconstruction. METHODS: In a retrospective study all patients with complex defect injuries of the upper extremity that were treated by V.A.C. prior to reconstruction between August 2003 and September 2005 were analyzed. RESULTS: 7 patients (6 male, 1 female, 14-70 years) were included in the study. The patients suffered from subtotal upper arm (n = 1) and forearm (n = 1) amputation, complex multilevel amputation injury of the forearm (n = 1), slash wound of the forearm with skin defect and discontinuity of all volar structures (n = 1), complex open forearm fractures with skin and soft tissue defects (n = 2), and almost complete necrosis of the flexor compartment following distal radius and proximal ulnar fracture and compartment syndrome (n = 1). Stabile defect coverage was achieved in all patients following V.A.C. therapy by myocutaneous free flaps (n = 2), split thickness skin grafts (STSG) (n = 2), sequential secondary suture (n = 1), and STSG + secondary suture (n = 2). Wound conditions improved significantly under V.A.C. therapy. 5 patients reported pain relief following induction of V.A.C. therapy. Due to reduction of tissue oedema secondary suture was facilitated in 3 patients. DISCUSSION: V.A.C. therapy represents an essential tool for treatment of complex injuries of the upper extremity with extended soft tissue defects. Decreased frequency of dressing changes as well as reduced tissue oedema considerably improved patient's comfort. Posttraumatic compartment syndrome or skin necrosis, which are often associated with macro amputations of the upper extremity, are efficiently treated with V.A.C., and secondary sutures may be performed despite initial skin defects.

[Vacuum-assisted closure (V.A.C.) of disastrous wound conditions in Madelung disease]. / Vakuumtherapie in der Behandlung desaströser Wundverhältnisse bei Morbus Madelung.

INTRODUCTION: A 54 year old patient presented with a Madelung disease. Symmetrically fatty deposits were present on both upper arms, at the abdomen and both thighs. Dermatitis was seen in the axillary fold at the skin bearing surfaces. Shoulder movement was impaired. Liposuction of both upper arms has already been performed, but no significant improvement was obtained. METHODS AND RESULTS: We performed a dermolipectomy of both upper arms. Postoperatively the patient suffered from a severe wound dehiscence with fulminant wound infection and septic clinical condition. Because of the large wound surface ((1/3) of the upper arm circumference) anemia occurred and had to be treated with blood transfusions. Wound exsudation caused a general hypoproteinemia. Multiple surgical debridements were necessary in order to clean the wound from necrotic tissue and afterwards moist dressings were applied. Furthermore the patient received intravenous antibiotic treatment. After stabilisation of the general condition of the patient and the wound situation a polyvinyl sponge and a vacuum-sealing were applied and continuous suction was performed (125 mm Hg). This resulted in a significant improvement of the wound situation in regards to wound cleaning, induction of granulation tissue, diminished wound size, reduction of oedema and pain reduction. After four weeks of vacuum therapy defect coverage was possible and split thickness skin grafting was performed. The skin graft was attached to the wound using a vacuum-dressing. After removal of the vacuum dressing on the fifth day the skin graft has completely healed. DISCUSSION: Vacuum therapy is suitable for disastrous wound conditions in order to achieve a defect size reduction, pain reduction and create a clean wound with granulation tissue for further surgical defect coverage using skin grafts or flaps.

[Reconstruction of tumor induced defects in head and neck surgery with individualized prefabricated three dimensional flaps with the use of continuous vacuum therapy]. / Rekonstruktion von tumorbedingten Defekten in der Kopf-Hals-Chirurgie mit individualisierten dreidimensionalen Lappen unter Anwendung der Vakuumtherapie.

Despite recent developments in oncologic head and neck surgery extensive tissue and functional defects following radical tumor resections remains a surgical challenge. Individually prefabricated free flaps to meet the needs of the functional defect can be generated with the help of continuous computer-assisted vacuum therapy. This accelerates the pre-fabrication of three-dimensional composite flaps and enhances therapeutic safety. Changes of the genuine structure of such flaps are induced by implantation of autologous or heterologous transplants alone or in combination with alloplastic materials as a supportive element. Application of vacuum therapy is useful to accelerate the process of pre-fabrication by the induction of angiogenesis. Besides this the continuous removal of wound exudate, possible seroma or haematoma and secondary compression of the different tissue layers improve the modelling of the three-dimensional construct. This article describes the technique of vacuum therapy for the pre-fabrication of three-dimensional pedicled or free flaps for reconstruction of defects after tumor resection in the head and neck area.

Causes behind a human cheese-borne outbreak of gastrointestinal listeriosis.

In a previous paper, we reported an outbreak of gastrointestinal listeriosis due to consumption of fresh cheese made from raw milk and manufactured on a summer farm. The aim of the present study was to investigate why the cheese harbored Listeria monocytogenes. To our knowledge, this is the first documented outbreak of listeriosis caused by raw milk cheese where the human epidemic strain has been cultured from a dairy animal, whose milk has been used for cheese production. The conditions on a summer farm can hardly fulfil the requirements for hygienic and strictly controlled conditions necessary for safe processing of fresh cheese.

Febrile gastroenteritis after eating on-farm manufactured fresh cheese--an outbreak of listeriosis?

An outbreak of febrile gastroenteritis affected consumers of on-farm manufactured dairy products from a summer farm in Sweden. Symptoms included diarrhoea, fever, stomach cramps and vomiting in 88, 60, 54 and 21% of cases identified. The median incubation period was 31 h. A cohort study with 33 consumers showed an attack rate of 52% and an association between the total amount of product eaten and illness (P=0.07). Twenty-seven of 32 (84%) stool samples cultured for Listeria monocytogenes tested positive, although there was no association between clinical disease and the isolation of L. monocytogenes. In addition, gene sequences for VTEC and ETEC were detected in 6 and 1 subjects, respectively. Bacteriological analysis of cheese samples revealed heavy contamination with L. monocytogenes and coagulase positive staphylococci in all of them and gene markers for VTEC in one of them. Molecular profiles for L. monocytogenes isolated from dairy products, stool samples and an abscess from 1 patient who developed septic arthritis were identical. Results of both microbiological and epidemiological analyses point to L. monocytogenes as the most likely cause of this outbreak. The finding of markers for VTEC in some humans and cheese samples means that a mixed aetiology at least in some cases cannot be conclusively ruled out.

Vasoactive intestinal peptide stimulation of human trabecular meshwork cell growth.

PURPOSE: To demonstrate that vasoactive intestinal peptide (VIP), a 28-amino acid neuropeptide, is a growth factor of human trabecular meshwork (TM) cells in culture and in a corneoscleral explant organ culture treated with laser trabeculoplasty (LTP). METHODS: Proliferating human TM cells in cell cultures were incubated with VIP for 20 hours, followed by total cell number determination, using a Coulter counter. The percentage of proliferating TM cells was assessed, using an antibody against the proliferating cell nuclear antigen (PCNA). To test the growth effect of VIP on TM cells in situ, corneoscleral explants in organ cultures were first treated with argon LTP to initiate TM-cell proliferation and then were exposed to VIP for 48 hours. The mitotic TM cells were demonstrated immunocytochemically, using anti-PCNA in paraffin sections of the explants; and the total number of TM cells was determined after paraffin sections were counterstained by hematoxylin. RESULTS: Vasoactive intestinal peptide dose-dependently stimulated the proliferation of TM cells in cell culture. Treatment with 5 x 10(-10) M VIP resulted in a maximal increase of 40% in cell number. The effect of VIP was blocked by a VIP antagonist. The number of PCNA-stained TM cells and the total cell number in the TM in LTP-treated corneoscleral explants were increased by VIP. CONCLUSIONS: Exogenously applied VIP stimulated the proliferation of human TM cells in subconfluent cultures and in LTP-treated corneoscleral explants. In that LTP has been shown to increase the number of TM cells in situ, the growth stimulatory effect of VIP may help enhance this therapy.