Dupuytren’s disease of the radial side of the hand

Dupuytren’s disease with involvement of the radial aspect of the hand is a clinical entity, which affects the thumb, the first web space, and the index finger. It can be primary or secondary. The palmar fascia anatomy and the clinical aspects of radial Dupuytren’s disease are described. The authors propose a new classification, which has the advantage in requiring only a clinical evaluation. One hundred and fifteen patients were examined for Dupuytren’s disease with radial involvement, primary or secondary, in the Hand Surgery Unit of the University of Modena between 1985 and 1994. Eighty-two (68.3%) of them were treated surgically as hand function was compromised by first web retraction. The most commonly used incisions of the first commissure are straight-line with Z-plasties or the Hirschowitz incision; either a zigzag incision or a Bruner incision is made over the thumb or index finger; Z-plasties described by Iselin are reserved for patients with severe contraction of the index finger. Results were assessed by improvement coefficient, calculated as the ratio of the difference between preoperative and postoperative scores to postoperative scores. Generally, Dupuytren’s disease with radial prevalence is not aggressive and progresses slowly and may be exclusively radial or associated with ulnar disease. In other patients, as the disease progresses, loss of function becomes apparent owing to retraction in adduction of the first commissure and flexion contracture of the thumb or index finger.     

Dupuytren’s disease of ring and little finger

Objective  Resection of fibrous digitopalmar connective tissues to improve finger function. Indications  Dupuytren’s disease in Tubiana’s stage >III. Partial fasciectomy: at any age, for localized involvement, unlimited degree of contracture, and for recurrences. Complete fasciectomy: extension lag <45° of an individual finger, favorable skin condition, and progressing Dupuytren’s disease. Contraindications  Poor general health. State after stroke. Infection or eczema of hand. Excessive alcohol intake. Uncooperative patient. Relative: HIV infection. Surgical Technique  Antegrade dissection of the fibrous tissue from palm to distal interphalangeal joint through a Y-shaped palmar incision or longitudinal digital incision interrupted by Z-plasties at the level of flexion creases. Dissection starts at the radial healthy side. For partial fasciectomy, the fibrous tissue is removed including a safety margin. During complete fasciectomy, the entire triangular palmar fascia including the vertical septae is excised. The so-called open palm technique is a particular form of a partial fasciectomy whereby transverse incisions are not closed as opposed to longitudinal incisions which are always sutured. Results  In a prospective study, 48/239 patients were followed up >2 years. Recurrence rate was 39.7% while hand function improved by 70–86% in ulnar type. Complications observed in a retrospective study (1982–1991) of 566 patients (two thirds partial, one third complete fasciectomy): wound healing disturbance 3%, hematoma 2.5%, vessel injury 1.2%, nerve injury 1.8%, infection 0.7%, persisting edema 0.7%, scar contracture 0.2%. 87% of all recurrences appeared within 24 months. Their incidence after 2–10 years varied after partial or complete fasciectomy between 12.5 and 66%, and 21.4 and 39.7%, respectively. Only a small percentage of these needed a revision.     

Phlegmonous-infection in first degree Dupuytren’s disease

Dupuytren’s disease belongs to the group of so called fibromatoses and is considered to be a non infectious disease. Since first degree Dupuytren’s disease is generally not operated upon, we want to report the unexpected finding of phlegmonous-abcessing infection in nodules in Dupuytren’s disease in a 61 year old patient, who was treated surgically because of complaining painful symptoms in first degree nodules in his palm without clinically visible signs of infection.     

Role of the HLA System in the Pathogenesis of Dupuytren’s Disease

Dupuytren's disease (DD) is a familial, fibroproliferative, irreversible, and progressive disease of the palmar fascia, yet with unknown etiology. However, there is compelling evidence which has consistently suggested a genetic ethiopathogenesis given the high occurrence among the Northern European extraction, familial nature, and demonstration of concordance in twins. DD is an incurable, recurrent, and potentially debilitating disease with limited and ineffective treatments. Although a number of possible candidate genes have been investigated including matrix metalloproteinases (MMPs) and transforming growth factor-beta (TGF-β) genes, as yet, no consistent genetic biomarker has been identified for DD. The highly polymorphic human leukocyte antigen (HLA) region is an ideal biomarker target. There have been some coherent data within the literature to suggest a genotype to phenotype association between certain HLA loci and a number of fibrotic disorders such as keloid and scleroderma, markedly with class II molecules and disease pervasiveness and clinical progression. The aim of this review, therefore, was to investigate the evidence indicative of both positive and negative associations between particular HLA alleles and DD. There is a clear association with specific HLA alleles and predilection or protection to DD, though there is a pressing need for further supportive data. The most promising of links to the HLA region in terms of a definitive genetic biomarker is with the class II HLA-DR loci. This paper presents a detailed account of the immunogenetic component of DD and explores the possible mechanisms of association between specific HLA molecules and susceptibility to DD.     

Castleman’s disease of the spleen

Introduction: Castleman’s disease (CD), also known as giant or angiofolicular lymphoid hyperplasia or lymphoid hamartoma, is a group of atypical lymphoproliferative disorders that share common lymph node histological features and may be localized either to a single lymph node (unicentric) or occur systemically (multicentric).

Patient and Method: Herein, we present a rare case of a of 75-year-old female patient who was referred to our department and after a thorough work-up, underwent splenectomy with synchronous resection of an accessory spleen, splenic artery lymph nodes, and splenic hilar lymph nodes due to splenic involvement in a multicentric CD.

Results: The pathology of the specimens led to the conclusion that it was a case of polycentric HHV-8-positive CD, affecting the spleen, the accessory spleen, and the lymph nodes.

Conclusions: Incidence of this rare condition is believed to be approximately 0.001–0.05%. CD has been linked to the human immunodeficiency virus (HIV), human herpes virus 8 (HHV-8), and is associated with malignancies. The pathogenesis mechanism is considered to be a dysregulation and hypersecretion of cytokines, either idiopathic or secondary to a viral infection, with the latter considered the most frequent. Solid organ involvement is very rare as is splenic involvement.     

Castleman’s disease of the pleura

Castleman’s disease is a rare benign disease of the lymph nodes. Its origin from the pleura is rare. Surgical excision, when feasible, appears to provide good results. We encountered a patient who had the disease arising from the parietal pleura. We present the clinical scenario, investigations, and our management of the patient.     

Examining the efficacy and maintenance of contracture correction after collagenase clostridium histolyticum treatment for Dupuytren’s disease

Background

This study examined the efficacy, complications, and contracture recurrence in patients who received injectable collagenase clostridium histolyticum (CCH) for Dupuytren’s-induced metacarpophalangeal (MP) and proximal interphalangeal (PIP) joint contractures.

Methods

A retrospective chart review at one center compared the degree of MP and PIP joint contracture pre-injection, post-cord rupture, and at final follow-up after a minimum duration of 6 months. Recurrence was defined as a 20 ° or greater increase in contracture above the minimum value achieved.

Results

Of 102 eligible patients, 48 patients (47 %) (31 males, 17 females) were available for review. 53 digits and 64 joints (46 MP joints and 18 PIP joints) were studied. The mean patient age was 66 years (range, 48–87 years) and mean follow-up duration was 15 months (range, 6 to 25 months). The mean MP joint contracture was 51 ± 20 ° at baseline, 4 ± 8 ° post-cord rupture, and 9 ± 15 ° at latest follow-up. The mean PIP joint contracture was 39 ± 23 ° at baseline, 14 ± 14 ° at cord rupture, and 29 ± 20 ° at latest follow-up. Of the 46 MP joints and 18 PIP joints, 11 MP (24 %) and 7 (39 %) PIP joints met the recurrence criteria. Of 102 patients, 1 patient had a small finger flexor tendon rupture.

Conclusions

Despite the dramatic initial reduction in contracture, recurrence developed in a high proportion of patients over the study period. While initially effective, CCH may not provide durable contracture reduction. However, CCH remains a viable nonsurgical treatment for Dupuytren’s disease.     

Calcium-dependent signaling in Dupuytren��s disease

Background  

Previous studies suggest that Dupuytren’s disease is caused by fibroblast and myofibroblast contractility. Cell contractility in smooth muscle cells is caused by calcium-dependent and calcium-independent signaling mechanisms. In the calcium-dependent pathway, calcium/calmodulin activates myosin light chain kinase (MLCK). In this study, the effects of calcium/calmodulin inhibition with the FDA-approved drug fluphenazine on Dupuytren’s fibroblast contractility and MLCK expression were tested.     

Percutaneous fasciotomy for the treatment of Dupuytren��s disease��a systematic review

Background  

Dupuytren’s disease is described as a thickening of the palmar fascia. It typically affects men of Northern European descent in their fifties. The disease process starts as a nodule at the distal palmar crease that progressively gives rise to a cord invading distally toward the finger. Historically, different treatments have been described. Our purpose was to perform a meta-analysis of the evidence published on the percutaneous fasciotomy (PCF) treatment.     

Surgical outcome of Dupuytren’s disease—no higher self-reported recurrence after segmental fasciectomy

A single-center survey on the patients’ perception of recurrence after Dupuytren’s surgery was conducted. To evaluate the impact of surgical techniques on self-reported recurrence rates, a retrospective analysis of 216 surgically treated patients with a minimum 2-year follow-up was conducted using a postal questionnaire. Reported recurrence rates were somewhat lower in segmental fasciectomy (43%), which was performed in 39% of the patients compared to an overall reported recurrence rate of 54% in all surgical procedures. In total fasciectomy with skin resection and grafting, patients reported a surprisingly high recurrence rate of 63%. By taking the prognostic value of diathesis into account, the difference in recurrence rates between surgical techniques was not statistically significant. Nevertheless, no higher recurrence risk in segmental fasciectomy was noticed and total fasciectomy with or without skin resection did not appear to guarantee indefinite results. At this point, surgical treatment in Dupuytren’s disease is confined to correcting contractures, without curing the patients. Therefore, unless segmental fasciectomy is not feasible due to the severity of the contractures, we suggest to always consider minimal invasive surgery as a surgical option in the treatment of Dupuytren’s disease.     

Revision of the Keller’s procedure – pilot study

Keller’s excisional arthroplasty has become a popular procedure for the treatment of osteoarthrosis of the first metatarsophalangeal joint, and exceptional rates of success have been reported in the literature. However, a number of reports on its limitations have also been published. We report our technique and the results of a pilot study in 3 patients for the revision of a failed Keller’s procedure. Arthrodesis of the first MTP joint was performed in patients after a Keller’s procedure had failed. A 1/3 semitubular plate was used to stabilize an interposition bone graft, between the first metatarsal and proximal phalanx. Postoperative follow-up ranged from 6 to 18 months (mean 8 months). Each patient reported excellent pain relief and return to full mobility.     

Castleman’s disease of the mesorectum: Report of a case

Castleman’s disease is a benign lymphoid proliferative disorder, which most commonly presents as a solitary mass in the mediastinum, although extrathoracic sites have been reported in the neck, axilla, pelvis, mesentery, pancreas, and retroperitoneum. We report a case of asymptomatic, isolated Castleman’s disease in the mesorectum, which is extremely rare. The patient was a 34-year-old woman who underwent investigations for vaginal spotting. A presacral mass was located on the left side of the rectum, 10 cm from the anal verge. Contrast-enhanced computed tomography showed a large, well-demarcated, strongly enhancing mass with internal radiating septa in the mesorectum. The mass was well circumscribed and isointense to muscle on T1-weighted magnetic resonance imaging, appearing as a slightly hyperintense mass on T2-weighted imaging. We performed laparoscopic mesorectal mass excision, and histological examination revealed features typical of the hyaline-vascular type of Castleman’s disease. Thus, when a mesorectal mass is being investigated, Castleman’s disease should be considered in the differential diagnosis.     

Crohn’s disease of the esophagus: Report of a case

(Received for publication on Jan. 18, 1999; accepted on Sept. 17, 1999)     

Expression of gap junction proteins connexins 26, 30, and 43 in Dupuytren’s disease

Background and purpose

Dupuytren’s disease (DD) is a benign fibroproliferative process of the palmar aponeurosis showing similarities to wound healing. Communication of cells involved in wound healing is mediated by the composition of gap junction (GJ) proteins. We investigated the expression of 3 GJ proteins, connexins 26, 30, and 43 (Cx26, Cx30, and Cx43) in DD.

Patients and methods

Fragments of Dupuytren’s tissue from 31 patients (mean age 56 (30–76) years, 24 male) were analyzed immunohistochemically and compared to control tissue for expression of the GJ proteins Cx26, Cx30, and Cx43 and also alfa-smooth muscle actin (α-SMA).

Results

14 of 31 samples could be attributed to the involutional phase (α-SMA positive) whereas 17 samples had to be considered cords in the residual phase (α-SMA negative). Expression of Cx26 and Cx43 was seen in 12 of the 14 samples from the involutional phase, and Cx30 was seen in 7 of these. Only 4 of the 17 samples from the residual phase showed any Cx, and there was none in the controls.

Interpretation

The high expression of GJ proteins Cx26, Cx30, and Cx43 in α-SMA positive myofibroblast-rich nodules, which are characteristic of the active involutional phase of DD, suggests that connexins could be a novel treatment target for the treatment of DD.Dupuytren’s disease (DD) is a benign progressive disease of the palmar aponeurosis that leads to a permanent and irreversible flexion contracture of the fingers. Various genetic aberrations (Dolmans et al. 2011) and environmental factors (Burge et al. 1997) have been linked to the development of DD. Local tissue damage can cause myofibroblast proliferation or tissue repair (Verjee et al. 2009, Shih and Bayat 2010). Because of the similarities, parallels have been drawn between DD and wound healing (Tomasek et al. 2002, Howard et al. 2004, Shih and Bayat 2010, Holzer et al. 2013). Myofibroblasts are present in both DD and in wound healing, and play an important role throughout the wound healing process, eventually causing a large deposit of collagen III (Shih and Bayat 2010).Wound healing is a complex, carefully regulated process requiring communication between different cell types. In normal tissues, fibroblasts are widely separated by extracellular matrix, but contact each other through elongated protoplasmic extensions. Communication is provided by gap junction (GJ) channels (Kumar and Gilula 1996, Mese et al. 2007, Churko and Laird 2013). These GJs allow the passage of small molecules, including ions and second messengers, between cells (Kumar and Gulila 1996). The protein subunits of GJ channels are called connexins. Their composition is important for their selectivity regarding passage of molecules and—as a result—communication between cells (Kumar and Gulila 1996). GJs have been shown to be important in cell proliferation, migration, and differentiation (Kumar and Gulila 1996, Mese et al 2007). In recent years, it has been shown that connexins play a critical role in wound healing, and Cx26, Cx30, and Cx43 are involved in this process (Brandner et al. 2004).Connexin expression changes in the different stages of wound healing, and this is important in regulation of the process. Cx26, Cx30, and Cx43 expression is rapidly downregulated at the wound edge within 6 h of wounding. In the subsequent stages, cells at the edges continue to be Cx26- and Cx30-negative whereas cells behind the edges show upregulation. The loss of Cx43 staining in all cells of the regenerating epidermis appears to be important for induction of human wound healing (Coutinho et al. 2003, Brandner et al. 2004, Davis et al. 2013). Additionally, connexins have recently been shown to directly control gene expression and cell migration (Kardami et al. 2007).3 connexins have been reported to have a role in human wound healing: Cx26, Cx30, and Cx43 (Brandner et al. 2004). We determined the expression of Cx26, Cx30, and Cx43 in Dupuytren’s tissue immunohistochemically, to investigate a possible involvement of GJ proteins in the pathogenesis of DD.     

The incidence of postoperative flare reaction and tissue complications in Dupuytren’s disease using tension-free immobilization

Purpose

Open fasciectomy represents a standard treatment of Dupuytren’s disease. Although patients are commonly immobilized in extension to prevent postoperative contracture formation, immobilizing the extremity under tension may precipitate a flare reaction and scar-related complications. This study explores the incidence of flare reaction and other complications with postoperative tension-free splinting after fasciectomy for Dupuytren’s contracture.

Methods

We retrospectively reviewed patients’ charts that consisted of 228 procedures in 191 patients who underwent surgery by the senior author between 2000 and 2010. Postoperative notes were reviewed for wound healing problems, scar appearance, flare reaction, and complications. The grading system defined by Evans et al. was used to standardize flare reaction and scar complications.

Results

Using tension-free splinting, the incidence of flare reaction was 3.5 % (8/228). The eight patients that had flare reactions had mild involvement, and no severe reaction was observed. Fifteen patients had hypertrophic scars, eight had hypersensitive scars, and six had recurrent contractures.

Conclusions

The incidence of flare reaction using tension-free immobilization postoperatively was low in our study. According to our findings, wound healing problems are rare when tensionless splinting is utilized. Type of study/level of evidence Case series, Level IV, Therapeutic study     

Gender Ratio of Dupuytren’s Disease in the Modern U.S. Population

Epidemiological studies conducted largely in northern Europe and Australia have shown that Dupuytren's disease is less common in women, with reported overall male-to-female ratios ranging from 3:1 to 9.5:1. Epidemiological data from other countries cannot be extrapolated to the modern U.S. population due to genetic and environmental differences between populations. The aim of this study was to determine the gender ratio in Dupuytren's disease in the Boston, MA area. We conducted a retrospective study of patients diagnosed with Dupuytren's disease at two large academic hospitals in Boston, MA between the years January 1995 and July 2006. To minimize variability introduced by clinical diagnosis, we also used internal billing records to identify a subset of patients who received fasciectomies for Dupuytren's disease during this period. A total of 1,815 patients (1,150 men, 665 women) were identified at our institutions with a clinical diagnosis of Dupuytren's disease, giving an overall male-to-female ratio of 1.7:1. Of these, 234 patients (176 men, 58 women) received fasciectomies performed by the two senior authors, resulting in a male-to-female ratio of 3.0:1. The male-to-female ratio for patients younger than 54 years of age was 4.0:1, and the ratio approached 1:1 with increasing age. The male-to-female ratio observed in our patient population was lower than those previously reported in the literature, particularly for patients younger than 54 years of age. This study indicates that large-scale epidemiological studies are needed to accurately report Dupuytren's disease in the modern U.S. population.     

Surgical management of Crohn’s disease

Introduction

Crohn’s disease is an inflammatory bowel disease that can affect the entire gastrointestinal tract. It is chronic and incurable, and the mainstay of therapy is medical management with surgical intervention as complications arise. Surgery is required in approximately 70% of patients with Crohn’s disease. Because repeat interventions are often needed, these patients may benefit from bowel-sparing techniques and minimally invasive approaches. Various bowel-sparing techniques, including strictureplasty, can be applied to reduce the risk of short-bowel syndrome.

Methods

A review of the available literature using the PubMed search engine was undertaken to compile data on the surgical treatment of Crohn’s disease.

Results and conclusion

Data support the use of laparoscopy in treating Crohn’s disease, although the potential technical challenges in these settings mandate appropriate prerequisite surgical expertise.     

Initial treatment of parkinson’s disease

Opinion statement Initial treatment of early idiopathic Parkinson’s disease (PD) begins with diagnosis based on clinical evaluation supplemented by laboratory studies and brain imaging to exclude causes of secondary parkinsonism. In most cases, testing is normal and the diagnosis of PD rests on clinical criteria. In patients with mild symptoms and signs, the diagnosis of PD may not initially be apparent, and follow-up evaluation is needed to arrive at a diagnosis. Once the diagnosis is made, pharmacologic treatment may not be the first step. First, patient education is essential, especially because PD is a highprofile disease for which information and misinformation are readily available to patients and families. Counseling concerning prognosis, future symptoms, future disability, and treatment must be provided. Questions from patients concerning diet, lifestyle, and exercise are especially common at this point. The decision of when to initiate treatment is the next major consideration. Much controversy but relatively little light has been brought to bear on this issue. L-dopa was the first major antiparkinson medication to be introduced and remains the “gold standard” of treatment. Next in efficacy are the dopamine agonists (DAs). A debate has raged concerning whether initial dopaminergic treatment should be with L-dopa or DAs. Physicians have been concerned about forestalling the appearance of dyskinesias and motor fluctuations, whereas patients have incorrectly understood that L-dopa and possibly other antiparkinson drugs have a finite duration of usefulness, making it important to defer treatment for as long as possible. This has created “L-dopa phobia,” which may stand in the way of useful treatment. In spite of this controversy, there is uniform agreement that the appropriate time to treat is when the patient is beginning to be disabled. This varies from patient to patient and depends on age, employment status, nature of job, level of physical activity, concern about appearance, and other factors. The choice of a specific drug is sometimes dictated by the patient’s symptoms. For example, L-dopa is preferable for severe akinesia, an anticholinergic may be useful when tremor is the most prominent symptom (especially in those aged younger than 70 years), and DAs may be indicated for younger patients, more prone to dyskinesias and fluctuations, with relatively mild symptoms. It is also important to manage non-motor symptoms in patients with early PD. Anxiety and depression are particularly common at this stage and may be presenting symptoms of PD. Where appropriate, counseling and/or treatment with anxiolytics and antidepressants should be considered.     

Operative trends and physician treatment costs associated with Dupuytren’s disease in Canada

PURPOSE:

To examine treatment trends and costs associated with Dupuytren’s disease (DD) in Canada.

METHODS:

Data regarding fasciectomies, fasciotomies and digit amputations performed for DD from 2005 to 2010 were extracted from the Canadian Institute for Health Information database. The data were analyzed according to year, sex and five-year age groups. The estimated annual physician reimbursement costs for DD in Ontario were calculated using Ontario Health Insurance Plan billing information and the 2010 Physician Schedule of Benefits.

RESULTS:

The number and rate of fasciectomies remained stable from 2005 to 2009 (mean of 4067 and 1.24 per 10,000, respectively), but increased in the 2009/2010 fiscal year (to 4458 and 1.32 per 10,000). The number of fasciotomies increased from 133 in 2005/2006 to 201 in 2008/2009, but dropped to 183 in 2009/2010. The mean number of amputations remained stable (12 procedures).The ratio of males to females undergoing fasciectomies remained stable (4:1). The highest rate of fasciectomies was performed for the age groups 65 to 69 years and 70 to 74 years. Estimated mean physician remuneration for DD in Ontario remained stable ($3.2 million per annum).

DISCUSSION:

The results regarding patient demographics are comparable with results from previous literature. There was a trend toward an increasing number of fasciectomies and fasciotomies annually, with fasciotomies increasing faster than fasciectomies, which is reflective of the aging population and the recent attention to fasciotomies in the literature. The present study was the first to investigate treatment trends and physician reimbursement costs for the management of DD in Canada.