Bilateral Ankle Fusion in Leak Syndrome Induced Deformity

Bilateral Ankle Fusion in Leak Syndrome Induced Deformity ABSTRACT INTRODUCTION: Systemic capillary leak syndrome is a rare condition of unknown etiology defined by sudden episode of hypotension, high hematocrit and low serum protein concentration due to capillary hyperpermeability. Several treatments have been published for this pathology and eventual compartment syndrome but not for the disabling consequences. This clinical case highlights the negative orthopaedic consequences of a severe systemic attack and reports a subsequent deformity treatment option that resulted in patient quality of life improvement. To our knowledge, orthopaedic surgery for systemic capillary leak syndrome induced foot and ankle deformity is presented for the first time. CASE PRESENTATION: A 40-year-old caucasian female patient was referred to our institution for bilateral foot and ankle deformity after compartment syndrome during systemic capillary leak syndrome attack. She presented a bilateral rigid equino-cavo-varus-adductus deformity causing walk impairment. Bilateral ankle fusion by retrograde nailing was performed in a two-staged procedure. Rigid claw toes were also surgically addressed in an additional procedure. Radiologically, apparent ankle fusion occurred at 3 months post-op in both sides. Deformity correction allowed the patient to regain walking capacity. A right ankle tibiotalar failed arthrodesis was noticed at 24 months post-op. Prophylactic human immunoglobulin therapy was initiated after the last surgery with no recurrent attack registered during the 38 months follow-up period. CONCLUSION: Bilateral ankle fusion improved this patient quality of life by restituting walking capacity and lowering the probability of lower limb deformity related ulcers and infections. Major and minor complications are also discussed. This report contributes to the global knowledge about this syndrome and elicits the importance of the induced deformities surgical correction. Keywords: systemic capillary leak syndrome, (SCLS), compartment syndrome, equino-varus, ankle fusion, ankle retrograde nailing, human immunoglobulin. INTRODUCTION Systemic capillary leak syndrome (SCLS), describedby Clarkson et al. in 1960 [1], is a rare condition defined by sudden episodes of hypovolemic shock, elevated hematocrit and hypoalbuminemia without albuminuria [2, 3]. The etiology is still unknown, although several possible triggers have been reported as upper respiratory tract infections, sustained physical effort and menstruation [1, 4]. It results in generalized or segmental edema and, in severe cases,compartment syndrome requiring emergent fasciotomy [2-5]. The sudden intracompartimental high pressure often originates vascular and neurological damage and consequent lower limb deformity [3, 6]. Prolonged hospital stay may also aggravate this condition with osteoporosis following a long-term immobilization or limb disuse. Deformity frequently leads to walk impairment and decreased quality of life [7]. A few retrospective studies report ankle fusion for correction of compartment syndrome induced deformity. None of them included SCLS etiology for compartment syndrome [6,7]. Several treatments have been published for SCLS and compartment syndrome [1-5] but not for the disabling consequences. To our knowledge, orthopaedic surgery for SCLS induced foot and ankle deformity is presented for the first time. CASE PRESENTATION A 40-year-old caucasian female patient was referred to our institution due to bilateral foot and ankle deformity. She had been previously diagnosed with systemic capillary leak syndrome after sudden episode of hypotension, high hematocrit and low serum protein concentration. This acute phase was managed by fluid resuscitation.The consequent generalized compartment syndrome was treated with extensive fasciotomies of both arms, forearms, hands, thighs and legs. She stayed three weeks in the intensive care unit and eight months hospitalized. Although an intense physical therapy program was applied, she was unable to walk and autonomy was limited to a wheelchair. On examination she presented a bilateral severe stiff equino-cavo-varus-adductus deformity with retraction due to subcutaneous and muscle scarring and neurological deficit. Claw hallux and lesser toes were also present bilaterally. Plantigrade weight bearing was not possible (Fig. 1). American Orthopaedic Foot and Ankle Society (AOFAS) score was 20 points. Plain radiographs showed bilateral equinovarus ankle deformity with supinated feet (Fig. 2). Achilles, posterior tibial and flexor digitorum longus tendons lengthening was performed by medial approach associated to right tibio-talocalcaneal arthrodesis with a retrograde locked intramedullary nail (PANTA ® IntegraTM) by lateral approach. Distal fibula bone was used as an autograft. At 8 weeks of follow-up, plastic surgery applied a free skin graft over a persistent post-operative medial blister (Fig. 3). Apparent fusion was obtained at three months post-operatively (Fig. 4). Six months after the initial surgery, fusion of the left ankle was performed in exactly the same fashion. Surgical wound dehiscence was the short-term complication and was resolved with dressing changes. Fusion was obtained at three months post-operatively. (Fig. 4). Fourteen months after the first surgery, we documented recurrent dorsal proximal interphalangeal (PIP) joints inflammation with shoe wear. Right foot Moberg osteotomy, resection arthroplasty of PIP joints and flexor tendon tenotomy of all the lesser toes were performed. Left foot Moberg osteotomy, PIP joints fusion of the 2nd and 3rd toes with intramedullary guide implant (Ipp-On ® IntegraTM), PIP joints resection arthroplasty of the 4th toe and flexor tendon tenotomy of all the lesser toes were performed. Failure of the left hallux Moberg osteotomy staple caused hardware removal at 6 weeks post-op (Fig. 5). The rehabilitation protocol included immediate weight-bearing with walking boots and lower limb drainage. Prophylactic human immunoglobulin therapy was initiated after the last surgery. She regained walking capacity with balanced shoes and returned to work four months after left foot surgery. AOFAS score was 61 points. No toes related complaints, pressure ulcers or terminal necrosis of the toes were found. Twenty-four months after initial surgery she complained of right ankle pain de novo with weight bear and local swelling. Radiologically, failed arthrodesis with tibiotalar and subtalar bone reabsorption, peri-implant proximal radiolucency and distal calcaneus screw fracture were shown (Fig. 6). No local drainage or blood test infection parameters were found. Only symptomatic treatment and nonsteroidal anti-inflammatory drugs were prescribed. At 38 months of follow-up the patient refers no significant right ankle pain and no SCLS attacks recurrence were registered. DISCUSSION This high-risk patients and the unpredictable course of the disease required a multidisciplinary treatment options discussion. The patient never accepted irreversible autonomy loss and was aware of all the possible negative surgery consequences when she signed the surgery consent. There are few treatment options for long-term sequelae of compartment syndrome that include arthroscopic assisted arthrodesis, fusion with external fixator or internal devices such as plates or intramedullary nails. Arthroscopic arthrodesis is an excellent option when sparing the soft tissues is needed, however, this correction is limited to mild deformities. External fixation represents a serious risk of pin tract infection with no consensus about prevention treatment [8] and therefore we advocate as a potential risk for SCLS relapse. Fusion with plate and screws implies a considerable aggression to the already damaged soft tissues, although it represents a high stiffness construct. The authors managed this clinical case as an equino-varus-cavo-adductus deformity after lower extremity compartment syndrome. Retrograde nailing avoided extensive striping and Berend et al. has demonstrated better biomechanics stiffness compared to crossed screws [9]. Wang et al. [6] described excellent satisfaction rate after retrograde nailing for lower extremity compartment sequelae. No tendinous transfers were considered due to neurological deficit, scarred muscles and stiff joints. Claw toes deformity has a high risk of pressure ulcers by shoe wear conflict. Local infection in SCLS patients may represent a potential trigger for relapse and therefore must be corrected. Considering the stiff interphalangeal joints, hallux claw deformity was managed by bilateral Moberg osteotomy. Lesser toes were addressed by resection arthroplasty of PIP joints with the exception of the stiffer left second and third toes that were fixated with an intramedullary guide implant [10]. Fusion rate of only 50% (1/2 ankles) was low compared to another published study that show rates around 91% of primary bony union after bilateral ankle arthrodesis. However, none of these patients had post-compartment syndrome ankle deformity and related sequelae [11]. With no signs of infection, late failed arthrodesis may be related to compartment syndrome, local vascular and neurological damage, technical error or even SCLS per se. Three minor complications were registered. One large posteromedial blister required skin grafting six weeks after right ankle procedure. A left ankle lateral surgical wound dehiscence healed by secondary intention only with dressing changes. Failure of left hallux Moberg osteotomy staple was managed by hardware removal. These complications are relatively common and have been previously described in foot and ankle deformity correcting arthrodesis [6, 12]. They are usually due to poor skin and vascular conditions. No delayed weight bearing was noticed in rehabilitation program due to prompt and successful treatment. Concerning deformity correction, the results were very satisfactory. The AOFAS score increased from 20 to a total of 61 points even after tibio-talocalcaneal arthrodesisthat does not allow any hind foot movement. Moreover, the painless plantar support permitted walking without crutches after 24 months confined to wheelchair. Returning to work was also an important milestone for this patient. At 38 months of follow-up the patient is now asymptomatic with minor local right ankle edema and no walking limitation. Radiologically the bone reabsorption appears to have stabilized. Anecdotal evidence has shown good results in ankle arthrodesis revision after compartment syndrome [6]. However, one must consider unpredictable consequences of fusion revision in a SCLS patient, including a severe attack during the anaesthesia or post-op infection with potential lethal consequences. This patient has been diagnosed with idiopathic form of SCLS. Although no direct cause was identified she had been taking azithromycin for a respiratory tract infection which may represent a potential trigger [4,13]. Monoclonal gammopathy was also present at follow-up lab blood tests as seen in more than 90% of cases [2, 4]. There are several possible prophylactic treatments, which include B2-agonists, thalidomide, calcium channel blockers and chemotherapy with limited evidence considering the rarity of the disease. Intravenous immunoglobulin was administered in the last 12 months of follow-up with no relapses adding to the hypothesis that this may represent an effective prophylaxis [4,14]. CONCLUSION Bilateral ankle fusion improved this SCLS patient functional outcome by restituting the walking capacity. One late failed arthrodesis was the major complication and may require a secondary procedure although the patient is currently asymptomatic. Four surgeries were performed with no SCLS attacks recurrence registered at 38 months follow-up period. Prophylactic human immunoglobulin may have contributed to the absence of relapses. Although surgical correction of SCLS skeletal deformities sequelae represents an orthopaedic challenge, we believe that this treatment should be considered in previously autonomous highly motivated active patients. REFERENCES 1. Clarkson B, Thompson D, Horwith M, Luckey EH. Cyclical edema and shock due to increased capillary permeability. Am J Med. 1960 Aug;29:193-216. http://www.sciencedirect.com/science/article/pii/0002934360900188 2. Atkinson JP, Waldmann TA, Stein SF, Gelfand JA, Macdonald WJ, Heck LW, Cohen EL, Kaplan AP, Frank MM. Systemic capillary leak syndrome and monoclonal IgG gammopathy; studies in a sixth patient and a review of the literature. Medicine (Baltimore) 1977 May;56(3):225-39. http://journals.lww.com/md-journal/Citation/1977/05000/SYSTEMIC_CAPILLARY_LEAK_SYNDROME_AND_MONOCLONAL.4.aspx 3. Sanghavi R, Aneman A, Parr M, Dunlop L, Champion D. Systemic capillary leak syndrome associated with compartment syndrome and rhabdomyolysis. Anaesth Intensive Care. 2006 Jun;34(3):388-91. http://www.aaic.net.au/document/?D=2005303 4. Gousseff M, Arnaud L, Lambert M, Hot A, Hamidou M, Duhaut P, Papo T, Soubrier M, Ruivard M, Malizia G, Tieulià © N, Rivià ¨re S, Ninet J, Hatron PY, Amoura Z. The systemic capillary leak syndrome: a case series of 28 patients from a European registry. Ann Intern Med. 2011 Apr 5;154(7):464-71. http://annals.org/aim/article/746914/systemic-capillary-leak-syndrome-case-series-28-patients-from-european 5. Kyeremanteng K, DEgidio G, Wan C, Baxter A, Rosenberg H. Compartment Syndrome as a Result of Systemic Capillary Leak Syndrome. Case Rep Crit Care 2016;2016:4206397. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5027304/ 6.   Wang X, Ma X, Zhang C, Huang JZ, Gu XJ, Jiang JY. Ankle fusion with a retrograde locked intramedullary nail for sequela of lower extremity compartment syndrome. Chin J Traumatol. 2012;15(3):140-4. http://www.sciencedirect.com/science/article/pii/S1008127515302911 7. Lintz F, Colombier JA, Letenneur J, Gouin F. Management of long-term sequelae of compartment syndrome involving the foot and ankle. Foot Ankle Int. 2009 Sep;30(9):847-53. http://fai.sagepub.com/content/30/9/847.abstract 8. Jennison T, McNally M, Pandit H. Prevention of infection in external fixator pin sites. Acta Biomater. 2014 Feb;10(2):595-603. http://www.sciencedirect.com/science/article/pii/S1742706113004790 9. Berend ME, A biomechanical comparison of intramedullary nail and crossed lag sure fixation for tibiotalocalcaneal arthrodesis. Foot Ankle Int. 1997; 18(10). https://www.ncbi.nlm.nih.gov/pubmed/9347301 10. Coillard JY, Petri GJ, van Damme G, Deprez P, Laffenà ªtre O. Stabilization of proximal interphalangeal joint in lesser toe deformities with an angulated intramedullary implant. Foot Ankle Int. 2014 Apr;35(4):401-7. http://fai.sagepub.com/content/35/4/401.long 11. Henricson A, Kamrad I, Rosengren B, Carlsson Ã…. Bilateral Arthrodesis of the Ankle Joint: Self-Reported Outcomes in 35 Patients From the Swedish Ankle Registry. J Foot Ankle Surg. 2016 Sep 7. pii: S1067-2516(16)30176-4. http://www.jfas.org/article/S1067-2516(16)30176-4/abstract 12. Asomugha EU1, Den Hartog BD, Junko JT, Alexander IJ. Tibiotalocalcaneal Fusion for Severe Deformity and Bone Loss. J Am Acad Orthop Surg. 2016 Mar;24(3):125-34. https://www.ncbi.nlm.nih.gov/pubmed/26829585 13. Amoura Z, Papo T, Ninet J, Hatron PY, Guillaumie J, Piette AM, Blà ©try O, Dequiedt P, Talasczka A, Rondeau E, Dutel JL, Wechsler B, Piette JC. Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment. Am J Med. 1997 Dec;103(6):514-9. http://www.amjmed.com/article/S0002-9343(97)00272-6/abstract 14. Marra AM1, Gigante A, Rosato E. Intravenous immunoglobulin in systemic capillary leak syndrome: a case report and review of literature. Expert Rev Clin Immunol. 2014 Mar;10(3):349-52. http://www.tandfonline.com/doi/full/10.1586/1744666X.2014.882771

Stanley Saitowitz :: essays papers

"THE ESSENTIAL MEDIUM OF ARCHITECTURE IS SPACE. A VOID TO BE FILLED WITH LIFE." (SAITOWITZ) THIS IS WHAT STANLEY SAITOWITZ HAS COME TO BELIEVE AFTER HIS 20 PLUS YEARS AS AN ARCHITECT. THERE IS NOT MUCH HISTORY RECORDED ABOUT THIS PECULIAR ARCHITECT. WHAT WE DO KNOW IS THAT HE IS A SOUTH AFRICAN JEWISH ARCHITECT. HE STARTED HIS CAREER OFF BUILDING SMALL HOMES IN SOUTH AFRICA. HIS MOST FAMOUS EARLY PROJECT IS THE TRANSVAAL HOUSE, FOR WHICH HE LATER WROTE A BOOK ON. ( A HOUSE IN TRANSVAAL, SAITOWITZ 1979) IN THE EARLY EIGHTYS HE MOVED TO THE LUSH HILLS OF NORTHERN CALIFORNIA WHERE MANY OF HIS MASTERPIECES RESIDE AS WELL. HIS WORKS ALTHOUGH SCATTER THE HILLSIDES IN NORHTERN CALIFORNIA, ARE ALSO SCATTERED ACROSS THE COUNTRY AS WELL AS INTERNATIONALLY. HIS PROJECTS SPAN IN CERTAIN PLACES SUCH AS BOSTON, COLUMBUS INDIANA, NEW YORK, SAN FRANSISCO AND THE BAY AREA, AND BERLIN. FROM WHAT WE KNOW SAITOWITZ ATTENDED THE UNIVERSITY OF WITWATERSRAND IN JOHANNESBURG, SOUTH AFRICA. SAITOWITZ’S ROLE MODELS INCLUDE VICTOR HUGO, SOL WEXNER, SIGMUND FREUD AND LE COURBUSIER. SAITOWITZ STATES THAT HE IS "ALWAYS DREAMING ABOUT WHAT LE CORBUSIER WOULD BE DOING TODAY." CORBUSIER’S INFLUENCE CAN BE SEEN IN MANY OF SAITOWITZ’S WORK INCLUDING EXPOSED CONCRETE SURFACES AND SEPERATED STAIR WELLS, AS WELL AS MANY SPACE USAGES AND CURTAIN WALL CONSTRUCTION. ONE OF MY FAVORITE PROJECTS OF SAITOWITZ’S IS THE HOLLOCAUST MEMORIAL. THIS MEMORIAL CONSISTS OF SIX GLASS TOWERS; EACH ETCHED WITH ONE MILLION NUMBERS, SYMBOLIZING THE 6 MILLION INNOCENT JEWS THAT PERISHED UNDER THE EVIL HANDS OF THE NAZIS. THIS IS LOCATED ALONG FREEDOM WAY IN BOSTON, MASSACHUSETTS. FOR THIS HE EARNED THE HENRY BACON MEDAL, AN HONOR ONLY GIVEN TO 8 PREVIOUS ARCHITECTS SINCE ITS INCEPTION IN 1966 AFTER THE ARCHITECT OF THE LINCOLN MEMORIAL. EACH OF THE BOHEMOTH GLASS TOWERS SITS ASIDE A PIT LIT WITH FIBER-OPTIC LIGHT AND A STEAM GENERATOR. SAITOWITZ STATES THAT "SOME PEOPLE THINK THE PITS OF FIRE SYMBOLIZE THE SIX NAZI DEATH CAMPS, OTHERS FEEL THAT THE WARM AIR RISING UP FROM THE GROUND IS LIKE THAT OF HUMAN BREATH AS IT PASSES THROUGH THE GLASS CHIMNEYS TO HEAVEN." THIS IS VERY SIGNIFICANT TO ME BECAUSE MANY OF MY FAMILY MEMBERS PERISHED LONG BEFORE I WAS BORN IN THE HANDS OF THE NAZIS. MY GRANDPARENTS STILL BEAR THE LIFELONG SCARS, THE TATTOO ENGRAVED ON THEIR ARMS ARE LIFE-LONG REMINDERS OF THE MASS DESTRUCTION OF LIFE THAT OCCURRED IN THE SECOND WORLD WAR.

Scarlett Letter Sin Essay

The Different Shades of Sins An Irish Proverb states â€Å"All Sins cast long shadows. † Throughout the novel, The Scarlet Letter, Nathaniel Hawthorne viewed the sins as a spectrum of many colors. The novel revolves around Hester Prynne, who is convicted of adultery in colonial Salem by the Puritan society. For her punishment, she is condemned to wear the red scarlet letter â€Å"A† on her chest as a permanent sign of her sin.Exposing to sin and the temptation of its concealment in capricious degrees, Nathaniel Hawthorne demonstrated the different shades of sins through the actions of several main characters, Hester Prynne, Arthur Dimmesdale, and Roger Chillingworth. In the beginning of the book, we learn Hester Prynne committed the sin of adultery. Although meant to crush Hester’s spirit, she is proud of her sin and becomes a strong, tough minded character. While the author Hawthorne has Puritan heritage, he portrays Hester as one of the most â€Å"able† of people in Salem because she confessed her sin.While the religious and high ranking citizens of Salem continue to ridicule her for being and adulteress, â€Å"many people refused to interpret the scarlet A by its original signification† (168). The product of this sin was Hester’s daughter Pearl. Unlike the way of the Puritans, Hester creates â€Å"beauty, shining through the gorgeous robes† (87) of Pearl, and thus Pearl stands out in crowds. To Hester, her sin was marrying someone she did not love, Roger Chillingworth. In the eyes of Hester and Dimmesdale, they are not â€Å"the worst sinners in the world.There is one worse than even the polluted priest! That old man's revenge has been blacker than my sin. He has violated, in cold blood, the sanctity of a human heart. Thou and I, Hester, never did so! † (225) Going against the heart is the greatest sin possible to Hester and Dimmesdale and their â€Å"sin† was between two people who truly loved e ach other. Reverend Arthur Dimmesdale is the father of Pearl and the one who committed the sin of adultery with Hester. As a man of high power in Salem, he does not confess the sin and the mental guilt would slowly kill him.Eventually, â€Å"his moral force was abased into more than childish weakness† (105) Through the ability Hester has shown and the miserable state of Dimmesdale, Hawthorne shows the power of confessing a sin. In an attempt to escape for their pasts, Hester, Pearl, and Dimmesdale plan to leave Salem. However, Roger Chillingworth, Hester’s legal husband, also catches a ride on the same boat. Returning to Salem, Hester, Dimmesdale, and Pearl go the scaffold, the place where Hester is originally condemned. In his dieing moments, Dimmesdale confesses his sin in front of the townspeople while also revealing a self inflicted â€Å"A† on his chest.Of all the sinners, Roger Chillingworth was the biggest. First, his sin of marrying a younger women and s ending her over sees causes the adultery claim. When he arrives in Salem and learns of this, he commits his biggest sin†¦ violating the human heart. Rather than Hester’s adultery being her sin, Hawthorne goes against Puritan values by making violating the heart Hester’s true sin. At first, Chillingworth asks Hester to â€Å"speak out the name of thy fellow-sinner and fellow-sufferer! † (56) When she refuses, Chillingworth becomes an evil man, becoming uglier by the day.This shows that an unconfessed sin will kill you. Eventually, he becomes aware of the adultery with Hester and becomes Dimmesdale’s â€Å"leech. † After Dimmesdale dies, Chillingworth dies because he has no reason to live anymore. Based on Puritan beliefs, sin is an inescapable aspect of life. Hawthorne shows that Dimmesdale and Chillingworth both die because of their hidden sin. Contrary to this Hester, who confessed, lives a long, happy life. Since sin is inescapable, the only way to make up for it is by confessing or else the sin swill shadow you forever.

Ladies Blackpool4life And Men Blackpool4life - 2341 Words

1. Executive summary Ladies Blackpool4life and Men Blackpool4life is a new proposed group weight management programme extended from the wellness service in Blackpool. It is a 12week group weight management programme with follow ups (drop in sessions or other ways of communications) at 6 months and 18 months. The dietitian will also offer additional support and motivation via phone calls, support groups and text messages. The service will be designed for overweight and obese patients (who are at the greatest risk in Blackpool) and each session will be gender specific to improve outcomes. It will be a multidisciplinary and integrated service including one hour of interactive dietetic advice delivered by band 5 dietitian followed by one hour†¦show more content†¦Option 2 was chosen as it was the only option which would improve service outcomes, meet the Heathy Weight Strategy for Blackpool, meet NHS planning framework, National Skills Framework and current NICE guidelines. The expected outcomes for this service are a weight reduction of 5-10% weight per participant with weight maintenance, reduced waiting times, reduced the high levels of high ‘Did Not Attend’ (DNA), reduce health inequalities in Blackpool and a reduction in obesity associated health condition. Overall this will contribute to a reduction of cost and burden to the health social care. The programme costing  £82.5 per person is considered cost effective according to NICE (2014b) as it is less than  £100 per person. 2. Current service Statistics show there were 140 500 people in Blackpool in 2014 with a large number of older people (over 65 years) which is predicted to increase in the next 20 years (JSNA, 2014). Table 1 describes the population profile compared to England and Wales. It shows a mainly white ethnic population with a high rate of obesity and poor lifestyle choices. The life expectancy, healthy life expectancy and disability fee expectancy is the lowest in England which is an indicator of poor health. There is a high disability rate in Blackpool (9.9%) which is twice the national average. There is also a higher learning disability prevalence in Blackpool (Blackpool Council, 2013).