Surgery for Hirschsprung's disease: comparison between the Duhamel method and the transanal endorectal pull-through based on 59 patients and a review of the literature

Background:¦Hirschsprung's disease (HSCR) is a congenital malformation of the enteric nervous system due to the¦arrest of migration of neural crest cells to form the myenteric and submucosal plexuses. It leads to an anganglionic intestinal segment, which is permanently contracted causing intestinal obstruction. Its incidence is approximately 1/5000 birth, and males are more frequently affected with a male/female ratio of 4/1. The diagnosis is in most cases made within the first year of life. The rectal biopsy of the mucosa and sub-mucosa is the diagnostic gold standard.¦Purpose:¦The aim of this study was to compare two surgical approaches for HSCR, the Duhamel technique and the transanal endorectal pull-through (TEPT) in term of indications, duration of surgery, duration of hospital stay, postoperative treatment, complications, frequency of enterocolitis and functional outcomes.¦Methods:¦Fifty-nine patients were treated for HSCR by one of the two methods in our department of pediatric¦surgery between 1994 and 2010. These patients were separated into two groups (I: Duhamel, II: TEPT), which were compared on the basis of medical records. Statistics were made to compare the two groups (ANOVA test). The first group includes 43 patients and the second 16 patients. It is noteworthy that twenty-four patients (about 41% of all¦patients) were referred from abroad (Western Africa). Continence was evaluated with the Krickenbeck's score.¦Results:¦Statistically, this study showed that operation duration, hospital stay, postoperative fasting and duration of postoperative antibiotics were significantly shorter (p value < 0.05) in group II (TEPT). But age at operation and length of aganglionic segment showed no significant difference between the two groups. The continence follow-up showed generally good results (Krickenbeck's scores 1; 2.1; 3.1) in both groups with a slight tendency to constipation in group I and soiling in group II.¦Conclusion:¦We found two indications for the Duhamel method that are being referred from a country without¦careful postoperative surveillance and/or having a previous colostomy. Even if the Duhamel technique tends to be replaced by the TEPT, it remains the best operative approach for some selected patients. TEPT has also proved some advantages but must be followed carefully because, among other points, of the postoperative dilatations. Our postoperative standards, like digital rectal examination and anal dilatations seem to reduce the occurrence of complications like rectal spur and anal/anastomosis stenosis, respectively in the Duhamel method and the TEPT technique.

Anal mucosectomy for haemorrhoids: should we start to speak Chinese?

AIM: Circular stapled mucosectomy is the standard therapy for the treatment of symptomatic third-degree haemorrhoids and mucosal prolapse. Recently, new staplers made in China have entered the market offering an alternative to the PPH stapling devices. The aim of this prospective randomized study was to compare the safety and efficacy of these new devices. METHODS: Fifty patients with symptomatic third-degree haemorrhoids were randomized to mucosectomy either by using stapler A (CPH32; Frankenman International Ltd, Hong Kong, China; n = 25) or stapler B (PPH03; Ethicon Endo-Surgery, Spreitenbach, Switzerland; n = 25). All procedures were performed by two experienced surgeons. After the stapler was fired by one surgeon, the other surgeon, who was blinded for stapler type, evaluated the stapler line. Postoperative outcome including pain, complications and patient satisfaction were analysed. RESULTS: Demographic and clinical features were no different between the groups. There was no significant difference regarding venous bleeding (P = 0.55), but arterial bleeding was significantly more frequent when stapler B was used (P < 0.001). This led to significantly more suture ligations (P = 0.002). However, no differences regarding operation time (P = 0.99), weight of the resected mucosa (P = 0.81) and height of the stapler line (anterior, P = 0.18; posterior, P = 0.65) were detected. Postoperative pain scores (visual analogue scale) and patient satisfaction were no different either (P = 0.91 and P = 0.78, respectively). No recurrence or incontinence occurred during follow-up. CONCLUSIONS: CPH32 required significantly fewer sutures for bleeding control along the stapler line after circular mucosectomy. However, operation time, rate of postoperative complications and patient satisfaction were similar in both groups.

Bladder cancer documentation of causes: multilingual questionnaire, 'bladder cancer doc'

There is a considerable discrepancy between the number of identified occupational-related bladder cancer cases and the estimated numbers particularly in emerging nations or less developed countries where suitable approaches are less or even not known. Thus, within a project of the World Health Organisation Collaborating Centres in Occupational Health, a questionnaire of the Dortmund group, applied in different studies, was translated into more than 30 languages (Afrikaans, Arabic, Bengali, Chinese, Czech, Dutch, English, Finnish, French, Georgian, German, Greek, Hindi, Hungarian, Indonesian, Italian, Japanese, Kannada, Kazakh, Kirghiz, Korean, Latvian, Malay, Persian (Farsi), Polish, Portuguese, Portuguese/Brazilian, Romanian, Russian, Serbo-Croatian, Slovak, Spanish, Spanish/Mexican, Tamil, Telugu, Thai, Turkish, Urdu, Vietnamese). The bipartite questionnaire asks for relevant medical information in the physician's part and for the occupational history since leaving school in the patient's part. Furthermore, this questionnaire is asking for intensity and frequency of certain occupational and non-occupational risk factors. The literature regarding occupations like painter, hairdresser or miner and exposures like carcinogenic aromatic amines, azo dyes, or combustion products is highlighted. The questionnaire is available on www.ifado.de/BladderCancerDoc.

Molecular and clinical characterization of 25 individuals with exonic deletions of NRXN1 and comprehensive review of the literature.

This study aimed to elucidate the observed variable phenotypic expressivity associated with NRXN1 (Neurexin 1) haploinsufficiency by analyses of the largest cohort of patients with NRXN1 exonic deletions described to date and by comprehensively reviewing all comparable copy number variants in all disease cohorts that have been published in the peer reviewed literature (30 separate papers in all). Assessment of the clinical details in 25 previously undescribed individuals with NRXN1 exonic deletions demonstrated recurrent phenotypic features consisting of moderate to severe intellectual disability (91%), severe language delay (81%), autism spectrum disorder (65%), seizures (43%), and hypotonia (38%). These showed considerable overlap with previously reported NRXN1-deletion associated phenotypes in terms of both spectrum and frequency. However, we did not find evidence for an association between deletions involving the β-isoform of neurexin-1 and increased head size, as was recently published in four cases with a deletion involving the C-terminus of NRXN1. We identified additional rare copy number variants in 20% of cases. This study supports a pathogenic role for heterozygous exonic deletions of NRXN1 in neurodevelopmental disorders. The additional rare copy number variants identified may act as possible phenotypic modifiers as suggested in a recent digenic model of neurodevelopmental disorders. © 2013 Wiley Periodicals, Inc.

Pulsatile tinnitus caused by an aneurysm of the transverse-sigmoid sinus: a new case report and review of literature.

A 28-year-old woman consulted for disabling pulsatile tinnitus. Clinical examination suggested a venous etiology. An aneurysm of the transverse-sigmoid sinus was identified on computed tomography angiography (CTA) and confirmed by digital subtraction angiography. Endovascular occlusion of the aneurysm with detachable coils permanently eliminated the bruit. Thus, this report is of a new case of pulsatile tinnitus caused by an aneurysm of the transverse-sigmoid sinus, with a focus on a literature review and etiopathophysiology. Embryological studies suggest that these aneurysms represent a partial remnant of the petrosquamous sinus.

Giant extra-hepatic thrombosed portal vein aneurysm: a case report and review of the literature.

BACKGROUND: Extrahepatic Portal vein aneurysm (EPVA) is a rare finding that may be associated with different complications, e.g. thrombosis, rupture, portal hypertension and compression of adjacent structures. It is being diagnosed more frequently with the advent of modern cross-sectional imaging. Our review of the English literature disclosed 13 cases of thrombosed EPVA. CASE PRESENTATION: A 50-years-old woman presented with acute abdominal pain but no other symptom. She had no relevant medical history. Palpation of the right upper quadrant showed tenderness. Laboratory tests were unremarkable. A computed tomography showed portal vein aneurysm measuring 88 × 65 mm with thrombosis extending to the superior mesenteric and splenic vein. The patient was treated conservatively with anticoagulation therapy. She was released after two weeks and followed on an outpatient basis. At two months, she reported decreased abdominal pain and her physical examination was normal. A computed tomography was performed showing a decreased thrombosis size and extent, measuring 80 × 55 mm. CONCLUSIONS: Although rare, surgeons should be made aware of this entity. Complications are various. Conservative therapy should be chosen in first intent in most cases. We reported the case of the second largest thrombosed extra-hepatic PVA described in the literature, treated by anticoagulation therapy with a good clinical and radiological response.

Advance care planning for institutionalised older people: an integrative review of the literature.

BACKGROUND: This integrative review of the literature describes the evolution in knowledge and the paradigm shift that is necessary to switch from advance directives to advance care planning. AIMS AND OBJECTIVES: It presents an analysis of concepts, trends, models and experiments that enables identification of the best treatment strategies, particularly for older people living in nursing homes. DESIGN: Based on 23 articles published between 1999 and 2012, this review distinguishes theoretical from empirical research and presents a classification of studies based on their methodological robustness (descriptive, qualitative, associative or experimental). RESULTS: It thus provides nursing professionals with evidence-based information in the form of a synthetic vision and conceptual framework to support the development of innovative care practices in the end-of-life context. While theoretical work places particular emphasis on the impact of changes in practice on the quality of care received by residents, empirical research highlights the importance of communication between the different persons involved about care preferences at the end of life and the need for agreement between them. CONCLUSIONS: The concept of quality of life and the dimensions and factors that compose it form the basis of Advance care planning (ACP) and enable the identification of the similarities and differences between various actors. They inform professionals of the need to ease off the biomedical approach to consider the attributes prioritised by those concerned, whether patients or families, so as to improve the quality of care at the end of life. IMPLICATIONS FOR PRACTICE: It is particularly recommended that all professionals involved take into account key stakeholders' expectations concerning what is essential at the end of life, to enable enhanced communication and decision-making when faced with this difficult subject.

Mixed medullary-papillary carcinoma of the thyroid: report of a case and review of the literature.

BACKGROUND: Mixed medullary-follicular thyroid carcinoma denotes a rare and heterogeneous group of tumors displaying morphological and immunophenotypical features of both origins within the same lesion. METHOD: We report a case of a 41-year-old woman with a lump in the right side of the neck, increasing in pain and size over several weeks. Serum levels of calcitonine (1140 ng/L) and carcinoembryonic antigen (288 microg/L) were very high. Fine-needle aspiration cytology suggested a diagnosis of medullary thyroid carcinoma. Total thyroidectomy, along with bilateral functional neck and mediastinal lymph-node dissection, were performed. RESULTS: The histopathological examination yielded a diagnosis of medullary carcinoma in the right thyroid lobe, closely intermingled with a nonencapsulated classical papillary carcinoma. One ipsilateral lymph node showed micrometastasis of the medullary counterpart. CONCLUSION: When compared with other cases reported in literature, this particular presentation should be recognized, if required, morphologic and functional criteria are used. The treatment is mostly surgical, driven by the medullary component. The presence of micrometastasis in 1 ipsilateral cervical lymph-node underlines the importance of cervicomediastinal lymph-node dissection and careful searching for metastatic disease.

Infection prothétique de hanche à Listeria monocytogenes [Prosthetic hip infection due to Listeria monocytogenes: review of the literature and case report].

With the ageing of the population, articular prosthetic replacements are becoming more and more frequent. One of the most feared complications is prosthetic infection, mostly due to bacteria of the cutaneous flora. Listeria monocytogenes is rarely the cause. This paper describes the management of a hip prosthetic infection due to Listeria monocytogenes. The patient was cured with antimicrobial therapy and a two-stage exchange. This case report creates an opportunity to review the literature in the aim of determining the risk factors and the optimal care.

Supervised injection services : what has been demonstrated? : a systematic literature review

BACKGROUND: Supervised injection services (SISs) have been developed to promote safer drug injection practices, enhance health-related behaviors among people who inject drugs (PWID), and connect PWID with external health and social services. Nevertheless, SISs have also been accused of fostering drug use and drug trafficking. AIMS: To systematically collect and synthesize the currently available evidence regarding SIS-induced benefits and harm. METHODS: A systematic review was performed via the PubMed, Web of Science, and ScienceDirect databases using the keyword algorithm [("SUPERVISED" OR "SAFER") AND ("INJECTION" OR "INJECTING" OR "SHOOTING" OR "CONSUMPTION") AND ("FACILITY" OR "FACILITIES" OR "ROOM" OR "GALLERY" OR "CENTRE" OR "SITE")]. RESULTS: Seventy-five relevant articles were found. All studies converged to find that SISs were efficacious in attracting the most marginalized PWID, promoting safer injection conditions, enhancing access to primary health care, and reducing the overdose frequency. SISs were not found to increase drug injecting, drug trafficking or crime in the surrounding environments. SISs were found to be associated with reduced levels of public drug injections and dropped syringes. Of the articles, 85% originated from Vancouver or Sydney. CONCLUSION: SISs have largely fulfilled their initial objectives without enhancing drug use or drug trafficking. Almost all of the studies found in this review were performed in Canada or Australia, whereas the majority of SISs are located in Europe. The implementation of new SISs in places with high rates of injection drug use and associated harms appears to be supported by evidence.

Viral Retinitis following Intraocular or Periocular Corticosteroid Administration: A Case Series and Comprehensive Review of the Literature.

Abstract Purpose: To describe viral retinitis following intravitreal and periocular corticosteroid administration. Methods: Retrospective case series and comprehensive literature review. Results: We analyzed 5 unreported and 25 previously published cases of viral retinitis following local corticosteroid administration. Causes of retinitis included 23 CMV (76.7%), 5 HSV (16.7%), and 1 each VZV and unspecified (3.3%). Two of 22 tested patients (9.1%) were HIV positive. Twenty-one of 30 (70.0%) cases followed one or more intravitreal injections of triamcinolone acetonide (TA), 4 (13.3%) after one or more posterior sub-Tenon injections of TA, 3 (10.0%) after placement of a 0.59-mg fluocinolone acetonide implant (Retisert), and 1 (3.3%) each after an anterior subconjunctival injection of TA (together with IVTA), an anterior chamber injection, and an anterior sub-Tenon injection. Mean time from most recent corticosteroid administration to development of retinitis was 4.2 months (median 3.8; range 0.25-13.0). Twelve patients (40.0%) had type II diabetes mellitus. Treatments used included systemic antiviral agents (26/30, 86.7%), intravitreal antiviral injections (20/30, 66.7%), and ganciclovir intravitreal implants (4/30, 13.3%). Conclusions: Viral retinitis may develop or reactivate following intraocular or periocular corticosteroid administration. Average time to development of retinitis was 4 months, and CMV was the most frequently observed agent. Diabetes was a frequent co-morbidity and several patients with uveitis who developed retinitis were also receiving systemic immunosuppressive therapy.