Esophagocardioplasty, vagotomy-antrectomy and Roux-en-Y gastrojejunostomy: indication in cases with severe esophageal motor disfunction.

Almost 10% of patients with Crest syndrome associated with severe gastroesophageal reflux and 5-10% of patients with failed cardiomyotomy for achalasia present with cardial or distal esophageal organic stricture. Some of these cases are poor risk patients for surgery and therefore the surgeon must offer a safe procedure with low morbimortality, keeping in mind the pathophysiological motor pattern of these patients.In order to treat the stricture to improve the esophageal transit we treated patients with esophagocardioplasty associated with vagotomy-antrectomy and Roux-en-Y gastrojejunostomy, thereby avoiding the potential acid or biliary reflux in poor risk patients in whom esophagectomy would be a very deleterious procedure. All four patients had a good postoperative evolution and late control demonstrated good esophagogastric transit with no postoperative esophagitis.

[LEFT ATRIAL APPENDAGE CLOSURE IN A PATIENT WITH SYSTEMIC SCLEROSIS]. / LE CAS CLINIQUE DU MOIS. Fermeture percutanée de L'auricule gauche chez une patiente souffrant de sclérodermie.

We report the clinical history of a 69 year-old female who suffered from systemic sclerosis and in whom we performed a percutaneous left atrial appendage closure due to recurrent gastrointestinal bleedings under anticoagulant therapy for chronic atrial fibrillation. We review the impact of scleroderma on the cardiac and digestive systems and discuss the issue of anticoagulation and its alternatives in uncommon clinical situations. We also describe the indications, technical aspects and potential complications of percutaneous left atrial appendage closure.

Telangiectasis in CREST syndrome and systemic sclerosis: correlation of clinical and pathological features with response to pulsed dye laser treatment.

Telangiectasia are cardinal features of systemic sclerosis (SS) and calcinosis, Raynaud's syndrome, esophageal motility, sclerodactyly, telangiectasias (CREST) syndrome. The etiology of telangiectasia in these syndromes is unknown, but vascular dysfunction has been proposed. However, the telangiectasia of CREST have anecdotally been considered relatively resistant to pulse dye laser (PDL), the treatment of choice for classic telangiectasia. The study was designed to test whether SS/CREST telangiectasia require more treatments than sporadic telangiectasia and to identify clinical and histological features that could explain such an effect. Nineteen skin biopsies from patients with SS or CREST and 10 control biopsies were examined and compared for features that may predict a differential response to PDL. Sixteen cases of SS or CREST treated with PDL between 1997 and 2007 were evaluated and response to treatment was compared with 20 patients with sporadic telangiectasis. Relative to normal skin, CREST/scleroderma telangiectasia exhibited thickened vessels in 17 out of 19 sections and thickened collagen fibers in the reticular or deep dermis in all sections. The number of treatments required to clear SS/CREST telangiectasia was approximately twofold higher. SS/CREST telangiectasia are more resistant to PDL but can be effectively cleared with more treatments.

Surgical management of digital calcinosis in CREST syndrome.

As a limited form of sclerodermy, CREST syndrome is characterized by calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia, which determine the acronym CREST. Calcinosis is a particularly difficult entity to treat given the paucity of effective options described in the literature. Treatment of finger calcinosis has a wide range of possibilities depending on the extent of calcifications and the involvement of deep structures. From a surgical point of view, whereas simple removal is adequate in minor outpatient cases, a radical debridement in the major and more painful cases seems required. A cover flap is needed particularly in the thumb due to its great functional importance, also if the fingertip is not involved. The authors recommend the kite flap for the dimensions, the tissue quality, and the possibility of giving sensation to the reconstructed area. With this surgical option, the transferred skin is soft, sensate, and the right fit. Usually, no further operations are needed for flap remodeling. The time required for sensory integration is about 2 years, often related to the age of the patient. Debridement and flap reconstruction usually give total resolution of pain, with complete recovery of thumb motion and the thumb-index finger grip.

Lung and heart-lung transplantation at University of Pittsburgh: 1982-2009.

The Lung Transplantation program at the University of Pittsburgh began in 1982. From the beginning to December 31, 2009, 1347, lung and heart lung transplantations have been done. (674 double-lung, 310 left single-lung, 227 right single-lung and 130 heart-lung transplantations. University of Pittsburgh maintains a data base, from the time of the inception of the program, of all recipients and donors. There is an increasing trend to do double-lung transplantation, as 5- and 10-year survival is better with double than single lungs. Our experience with heart-lung transplantation is considerable. The 4 most common indications for lung transplantation are: chronic obstructive disease (COPD), idiopathic pulmonary fibrosis (IPF), cystic fibrosis (CF) and pulmonary arterial hypertension. (PAH). Potential recipients are evaluated over 2 weeks. There is also a pathway for accelerated evaluation of sicker patients. Our recipient criteria are expanded and flexible; as many patients, who have been denied lung transplantation, at other major centers have been successfully transplanted by us with good outcomes. The median waiting time on the list in 2009 was 37 days. Our altruistic flexibility in recipient selection, balanced with respect to the altruistic gift of donor families, has been described in considerable detail. To solve the problem of shortage of donor lungs, we have expanded our donor selection criteria beyond the historic ideal donor, without comprising on outcomes. These selection criteria and our donor-lungs management protocol are also described in reasonable detail. We started using lungs from donors after cardiac death (DCD) in January 2007. Recently we reviewed our experience and literature, and devised a protocol; which is given in a robust table. We also participate as faculty in the educational program initiated by the Organ Procurement Organizations (OPO) of Pennsylvania. During procurement we use 500 microg of prostaglandin E-1 into the pulmonary artery just before X clamp, Perfedex infusion after X-clamp, at 70 ml/kg, with 500 microg of prostaglandin and 50 mg of nitroglycerine in the first bag, and retrograde flush of 500 ccs in each pulmonary vein. We have refined our recipient operation, from clam shell incision to bilateral antero-axillary incisions, preserving the sternum and internal mammary arteries, for both double and single-lung transplantation, with good outcomes. This technique and results have been described in generous detail. We use pneumoplegia, similar to cardioplegia, to protect the allograft from ischemic and reperfusion injury (Appendix III). Our technique of bronchial anastomosis and intraoperative management of septic lung disease has remained unchanged. Post-operatively we continue to use the ventilatory management of low FiO2 and high PEEP. Our immunosuppression and infection prophylaxis protocol is the same since 2003, when we started using Alemtuzumab (Campath) for induction, with minimization of the use of steroids. For maintenance, we use Tacrolimus and Mycophenolate Mofetil. Now, we also monitor the functional activity of the T cell by Cylex ImmuKnow. Lowered activity (< 100 ng/ml) suggests an increased risk of infection; and higher activity (> 200 ng/ml) suggests greater risks for rejection. Although we have expanded our recipient and donor pools, our outcomes have continued to improve. The overall survival of double-lung transplantation from 2003-2009 was 82.8% at one year and 56.8% at 5 years. This compares well with the international data which shows an overall survival rate of 80% at one year and 56% at 5 years. Results of lung transplantation will continue to improve, with our increasing understanding of mechanisms and management of ischemic-reperfusion injury, acute rejection and bronchiolitis obliterans syndrome.

[Manifestations of scleroderma at the hand--options for hand surgery in an interdisciplinary concept]. / Handbeteiligungen im Rahmen der progressiven systemischen Sklerodermie--Möglichkeiten der Handchirurgie im interdisziplinären Konzept.

Clinical manifestations of scleroderma at the hand include Raynaud's phenomenon, calcinosis cutis, sclerodactylia and teleangiectasia. With the progression of the disease, cutaneous and joint contractions, acro-osteolysis, necrosis of the finger tips, and even extensive digital ulceration are likely to occur. These painful and often rapidly advancing lesions cause loss of function and disfigurement and, untreated, often lead to mutilation of the affected hand. Only an interdisciplinary management including the hand surgeon, the rheumatologist, and the physiotherapist can guarantee optimal treatment. Drug therapy should be included as well as physical therapy. Both should be made use of before and accompanying surgical treatment. Surgical therapy consists of treatment of the infections, excision of calcinosis, arthrodesis, in particular of the proximal interphalangeal joints, and sympathectomy. Amputation remains a final option, whereas with timely and sufficient treatment, amputations can be avoided and an improvement of function and an alleviation of the symptoms can be achieved. Among the non-operative treatment options, behavioural training, calcium antagonists, prostacyclin derivatives, topical nitrates as well as plexus anesthesia and stellatum blocks have proved to be effective. Recent drug therapies include endothelin-receptor antagonists for the prevention of digital ulceration and phosphodiesterase-V antagonists in treatment of Raynaud's phenomenon and induction of ulcer healing. With reference to several cases seen at our institution, we propose an interdisciplinary treatment concept for acral manifestations of scleroderma.

Surgical management of the hand in scleroderma.

Disabling deformity of the hand is a hallmark feature of the person afflicted with scleroderma. However, existing literature provides little guidance to operative treatment for the wide spectrum of hand derangement. Although arthrodesis is generally recommended for severe flexion contractures of the interphalangeal joints, other surgical procedures such as arthroplasty, excision of painful calcinosis, and digital sympathectomy have been employed sparingly, undoubtedly due to potentially hazardous soft tissue conditions. Based on experience with 70 scleroderma patients requiring 272 hand operations, this article provides further insight as to the role of surgical treatment for the scleroderma hand. The favorable results in this relatively large series of cases support the efficacy of precisely timed and skillfully executed surgery in the alleviation of pain, prevention of tissue loss, preservation of function, and improvement in aesthetics. For the ischemic tissues of the scleroderma hand the prerequisite for uncomplicated surgery is a tension-free wound, often requiring judicious skeletal shortening and healing by secondary intention.

Limited microsurgical arteriolysis for complications of digital vasospasm.

Twenty-two digits in seven patients with chronic digital vasospasm were surgically treated after failed medical management. All patients complained of severe ischaemic pain. Chronic digital tip ulceration was present in seven digits and dry gangrene in one. Following surgical microarteriolysis all digital ulcers healed completely. Severe digital ischaemic pain was significantly improved in all digits and completely resolved in 19 of 22 digits.

[Argon plasma coagulation in endoscopic therapy of CREST syndrome associated upper gastrointestinal hemorrhage]. / Die Argonplasmakoagulation in der endoskopischen Therapie einer CREST-Syndrom-assoziierten oberen gastrointestinalen Blutung.

We report on the case of a 55-year-old patient suffering from progressive systemic sclerosis (PSS). The patient was sent to our department when clinical symptoms of an acute upper gastrointestinal hemorrhage occurred. Upper endoscopy showed a watermelon stomach and fresh blood in the stomach. The presence of teleangiectasias in the antrum could be proved histologically. Since the teleangiectasias found in the antrum were the only possible source of the hemorrhage three sessions of endoscopic argon plasma coagulation were performed. Macroscopically, a nearly complete disappearance of teleangiectasias could be achieved. After a follow-up of six months, there have been no clinical signs of another hemorrhage episode. This case shows that the existence of gastrointestinal teleangiectasias should be considered when chronic anemia or acute gastrointestinal hemorrhage occur in patients with PSS. Further it is demonstrated that even extended gastrointestinal teleangiectasias can be successfully treated by endoscopically performed argon plasma coagulation.

[Hypercalcinosis of the hands in scleroderma. 2 case reports]. / Hyperkalzinose der Hände bei Sklerodermie. Zwei Fallbeschreibungen.

Hypercalcinosis of the hand in association with scleroderma represents a rare and benign soft-tissue calcification. Reported are two cases of symmetric affection of soft-tissue layers of both hands in association with scleroderma. Superinfection and exulcerations necessitated surgical treatment. Diagnosis, therapy, and progress are described.