Bilateral Ankle Fusion in Leak Syndrome Induced Deformity

bilaterally symmetrical ankle nuclear alinement reaction in escape valve Syndrome Induced De classityABSTRACTINTRODUCTION Systemic capillary making water syndrome is a rare condition of unknown etiology defined by sudden episode of hypotension, high hematocrit and low serum protein slow-wittedness due to capillary hyperpermeability. Several discourses have been make for this pathology and planetual compartment syndrome provided not for the disabling consequences. This clinical causal agency highlights the negative orthopaedic consequences of a severe general encounter and breeds a subsequent stain treatment option that resulted in unhurried quality of living improvement. To our knowledge, orthopaedic surgical process for systemic capillary escape valve syndrome induced buttocks and mortise joint blur is presented for the first time.CASE PRESENTATION A 40-year-old Caucasic female patient was referred to our institution for bilateral foot and articulatio taloc ruralis deformity later compartment syndrome during systemic capillary leak syndrome attack. She presented a bilateral rigid equino-cavo-varus-adductus deformity make walk impairment. Bilateral mortise joint fusion by retrogressive nailing was performed in a two-staged procedure. Rigid claw toes were also surgically addressed in an additional procedure. Radiologically, apparent mortise-and-tenon joint fusion occurred at 3 months post-op in both sides. Deformity rectification allowed the patient to regain walking capacity. A ripe(p) ankle tibiotalar failed arthrodesis was noticed at 24 months post-op. preventative piece immunoglobulin therapy was initiated later on the last surgery with no recurrent attack registered during the 38 months action period.CONCLUSION Bilateral ankle fusion amend this patient quality of life by restituting walking capacity and heavy(a) the probability of swallow weapon system deformity connect ulcers and infections. Major and utility(pren ominal) complications are also discussed. This report contributes to the global knowledge around this syndrome and elicits the sizeableness of the induced deformities surgical correction.Keywords systemic capillary leak syndrome, (SCLS), compartment syndrome, equino-varus, ankle fusion, ankle retrograde nailing, human immunoglobulin.INTRODUCTIONSystemic 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(prenominal) assertable knowledgeabilitys have been reported as upper respiratory tract infections, preserve fleshly effort and menstruation 1, 4. It results in generalized or metameric edema and, in severe cases,compartment syndrome requiring emergent fasciotomy 2-5. The sudden intracompartimental high pull often originates vascular and neurological damage and consequent lower weapon system deformity 3, 6. Prolonged hospital stay may also exasperate this condition with osteoporosis following a long immobilization or limb disuse. Deformity frequently leads to walk impairment and decreased quality of life 7.A fewer 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 PRESENTATIONA 40-year-old caucasian female patient was referred to our institution due to bilateral foot and ankle deformity. She had been antecedently 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 consequ ent generalized compartment syndrome was treated with elongated fasciotomies of both arms, forearms, hands, thighs and legs. She stayed collar 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 abjuration due to subcutaneous and muscle scarring and neurological deficit. Claw great toe and lesser toes were also present bilaterally. Plantigrade freight bearing was not affirmable (Fig. 1). American Orthopaedic Foot and Ankle Society (AOFAS) check off 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 ivory was utilise as an autograft. At 8 weeks of follow-up, plastic surgery applied a free discase 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 leftover over(p) ankle was performed in exactly the selfsame(prenominal) 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 scrolled recurrent dorsal proximal interphalangeal ( slur) joints inflammation with shoe live on. Right foot Moberg osteotomy, resection arthroplasty of fritter 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 enter (Ipp-On IntegraTM) , PIP joints resection arthroplasty of the quaternate toe and flexor tendon tenotomy of all the lesser toes were performed. Failure of the left hallux Moberg osteotomy basic caused hardware removal at 6 weeks post-op (Fig. 5). The replenishment 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 match shoes and returned to work four months after left foot surgery. AOFAS score was 61 points. No toes related complaints, pressure ulcers or terminal slough of the toes were found.Twenty-four months after initial surgery she complained of right ankle offend 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 inventory test infection parameters were f ound. 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.DISCUSSIONThis high-risk patients and the unpredictable course of the disease affectd a multidisciplinary treatment options discussion. The patient neer accepted irreversible autonomy loss and was aware of all the likely 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 comminuted option when economical the soft tissues is needed, however, this correction is limited to mild deformities. External fixture represents a serious risk of pin tract infection with no consensus about prevention treatment 8 and there fore we advocate as a capableness 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 effectiveness trigger for relapse and therefore must be corrected. Considering the stiff interphalangeal joints, hallux claw deformity was managed by bilateral Moberg osteoto my. 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 other 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 pip-squeak 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 run and successful treatment.Concerning deformity correction, the results were very satisfactory. The AOFAS score change magnitude from 20 to a total of 61 points even after tibio-talocalcaneal arthrodesisthat does not allow some(prenominal) 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 shew has shown good results in ankle arthrodesis revision after compartment syndrome 6. However, one must consider unpredictable c onsequences 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 neckcloth 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.CONCLUSIONBilateral 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 extremely motivated active patients.REFERENCES1. Clarkson B, Thompson D, Horwith M, Luckey EH. Cyclical edema and shock due to increased capillary permeability. 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