Ilizarov treatment of congenital pseudarthrosis of the tibia clinics in orthopedic surgery vol. Periosteal grafting for congenital pseudarthrosis of. This incorrect bone healing is referred to by orthopedists as nonunion. Tibial pseudarthrosis is the name given to a fracture of the tibia the larger of the two bones in the lower leg that does not heal correctly. The differential diagnosis of congenital pseudarthrosis of. Jun 22, 2019 kesireddy n, kheireldin rk, lu a, cooper j, liu j, ebraheim na. Five cases of congenital pseudarthrosis of the tibia successfully treated by a free. Ilizarov treatment of congenital pseudarthrosis of the.
Classifications of radiographic findings include atrophic or hypertrophic pseudarthosis as well as cystic or dystrophic lesions. Ilizarov treatment of congenital pseudarthrosis of the tibia. Congenital pseudarthrosis of the tibia cpt is a rare pathology, which is. Examination revealed a varus deformity of the left leg, which was 5 cm shorter than the right. A retrospective study of surgical treatment of congenital. There are at least four dysplastic lesions of the congentially bowed tibia with or without cysts which are still being confused in the literature, but which have quite different behaviour patterns. The pseudarthrosis is usually not present at birth and.
A thickened cuff of periosteum and interruption of the bone compacta are noted. Dec 28, 2015 the study aims to evaluate the clinical result of combined surgery in management of congenital pseudarthrosis. Congenital pseudarthrosis of the fibula is rare with only 15 cases reported in the literature. Other methods include direct current or pulsed electromagnetic field, ipsilateral transfer of the fibula or contralateral free vascularized fibular transfer, circular. Pseudarthrosis fibula accompanies or precedes same condition in ipsilateral tibia. Jennifer is a 40 yo from atlanta, ga who had a scoliosis fusion done many years ago with sublaminar wires and rods, and then had an extension fusion done complicated by. Tibial pseudarthrosis occurs in association with neurofibromatosis in 55% of patients, although only 6% of patients with neurofibromatosis have a pseudarthrosis. Of all the diseases in children, congenital pseudarthrosis of the tibia cpt is probably one. Even when union is achieved, the residual deformities. Free vascular bone transfer has been reported in congenital pseudarthrosis of the tibia weiland and.
The key to the solution of this problem lies in the differential diagnosis. These goals can be achieved with intramedullary nailing, free vascularized fibular grafts, and external ringed fixators. Springer nature is making coronavirus research free. The study aims to evaluate the current surgical treatment and its result for congenital pseudarthrosis of tibia cptin children in china. Current treatment of congenital pseudarthrosis of the tibia. Pseudarthrosis of the spine is also referred to as a nonunion and means false joint. Congenital pseudarthrosis of the tibia paley institute. For example, if the shinbone, the tibia, does not heal after a fracture, a nonunion develops.
Congenital pseudarthrosis of the tibia cpt is difficult to treat in pediatric patients. There are also six different types of true congenital pseudarthrosis which exhibit similar differences in behaviour and. Although pseudarthrosis has been described in association with neurofibromatosis, the mechanism by which it arises has not been explained. Free vascularized fibular graft for tibial pseudarthrosis in. Between 1992 and 2007, nine patients with cpt were treated consecutively at our clinic with free fibula transfer. The surgical procedures most used for treating congenital pseudarthrosis of the tibia are intramedullary nailing associated with bone grafting, vascularized fibular graft and the ilizarov external circular fixator.
Additionally, external fixation may benefit patients by avoiding the donor site morbidity of contralateral free fibular harvest 22. Other methods include direct current or pulsed electromagnetic feld, ipsilateral transfer of the fbula or contralateral free vascularized fbular transfer, circular external fxation, im rodding, and combined external fxation and im rodding. Congenital pseudarthrosis of the tibia is a rare pediatric disorder that often presents with anterolateral bowing of the tibia. Jan 08, 2017 definition pseudarthrosis is a false joint associated with abnormal movements at the site 3. The ipsilateral fibula was transferred, and its vascular pedicle left intact and secured to both fragments of the tibial pseudarthrosis by cerclage wires. Congenital pseudarthrosis of the tibia cpt is an uncommon disease with various clinical presentations ranging from simple anterolateral tibial angulation to complete nonunion with extensive bone defects. Treat the congenital pseudarthrosis of the tibia with ilizar. The combined surgery includes sleeve resection of the pathological soft tissues, retrograde intramedullary rodding, packaged lilac bone autograft,and appliance of llizarov external fixation device. Proximal location of congenital pseudarthrosis of the tibia cpt is uncommon, and its management challenging, risking to end in amputation. Management of congenital pseudoarthrosis of the tibia and fibula. The split is created in such a way that it does not fracture either arm of the split. Congenital pseudarthrosis of the tibia is a rare disorder, 1 per 190 000 live births and is of unknown aetiology 1. Congenital pseudarthrosis of the tibia cpt refers to nonunion of a tibial fracture that develops spontaneously or after a minor trauma.
The full text of this article is available in pdf format. The pseudarthrosis is usually not present at birth and therefore is not truly congenital but occurs during the. Five cases of congenital pseudarthrosis of the tibia successfully treated by a free vascularised fibular graft are described. Apr 21, 20 the term pseudarthrosis is used commonly in the situation of a fracture nonunion. This study assessed the outcomes, at skeletal maturity, for 34 patients in whom congenital pseudarthrosis of the tibia cpt had been treated with intramedullary im rod fixation. Rose, treatment of congenital pseudarthrosis of the tibia. We believe this salvage method is superior to others not only because of the successful results, but also. Anteroposterior xrays revealed distal tibia and fibula fractures figure 1 with associated. We here report a case of proximal cpt managed in a limbsparing perspective and followed up until the end of growth. It occurs in both adults and children, and the paucity of descriptions for all types of the condition in the majority of textbooks bespeaks its comparative rarity.
Congenital pseudarthrosis of the tibia sciencedirect. Eventually the nonunion will develop into a pseudarthrosis related to motion between the bone ends. Excision of sclerotic ends followed by bone grafting free vascularised fibular grafts or bone transport with an ilizarov ring fixator. Pseudarthrosis of the forearm congenital hand and arm. Te pseudarthrosis of the tibia is separated apart by removing any fbrous tissue between the bone ends. Vascularized free fibular bone graft in the management of. A treatment regime using electrical stimulation in association with a variety of surgical procedures has improved the prognosis in congenital pseudarthrosis of the tibia one of the most challenging of all orthopaedic disorders. A fracture with nonunion generally forms a structural resemblance to a fibrous joint, and is therefore often called a false joint or pseudoarthrosis the greek stem pseudo means false and arthrosis means joint. Exact cellular mechanism is not known and despite a definite clinical relationship with neurofibromatosis, a cellular cause and effect has not been proven. Followup ranged from 5 to 34 months with a mean of 17. What does a spine pseudarthrosis pseudoarthrosis look. Congenital pseudarthrosis of the tibia presents one of the most difficult treatment challenges to the orthopedist. David feldman live talking about congenital pseudarthrosis of the tibia for fridays with feldman.
Nonunion is permanent failure of healing following a broken bone unless intervention such as surgery is performed. What does a spine pseudarthrosis pseudoarthrosis look like and why is it painful. Congenital pseudarthrosis of the tibia continues to pose one of the most difficult problems in paediatric orthopaedic surgery. Once disengaged the proximal and distal tibia will overlap relative to each other by straightening the bone ends. Pseudarthrosis and neurofibromatosis jama surgery jama. Between 40% and 80% are associated with neurofibromatosis. Congenital pseudoarthrosis of the tibia radiology reference. Congenital pseudarthrosis of the tibia cpt is a nonunion of a tibial fracture often occurring spontaneously or as a result of minor trauma. Cho has recommended that the last dooley category, which includes pseudoarthrosis of the tibia, be categorized as a class of tibial pseudoarthrosis, given that the treatment and outcomes of those patients are more dependent upon the tibial condition. Pseudarthrosis can occur at any place where spinal fusion was attempted and presents as either axial neck or back or radicular arms or leg pain that occurs months to years after a previous lumbar fusion. The natural history of the disease is extremely unfavorable and once a fracture occurs, there is a little or no tendency for the lesion to heal spontaneously. Congenital pseudarthrosis of the tibia cpt is a rare pathology, which is usually associated with neurofibromatosis type i. A detailed history was taken and there was no suspicion of physical abuse. Pdf treatment of congenital pseudarthrosis of the tibia.
The tibia resembles the oldfashioned onepiece wooden clothes pins. The technique consists of correction of the tibial deformity, intramedullary fixation and cancellous bone grafting, augmented by electrical stimulation using an. The effect of combined surgery in management of congenital. Pdf congenital pseudarthrosis of the tibia is a rare disease with a. The results in skeletally mature patients in whom type4 cpt had been treated with an im rod at an average of 11. Jul 06, 2011 congenital pseudarthrosis of the tibia cpt is an uncommon disease with various clinical presentations ranging from simple anterolateral tibial angulation to complete nonunion with extensive bone defects. Free vascularized fibular graft for tibial pseudarthrosis in neurofibromatosis herman h. Congenital pseudarthrosis of the tibia is a rare disease with a variable history. Grades of severity bowing of fibula without pseudarthrosis fibular pseudrthrosis without ankle deformity fibular pseudarthrosis with ankle deformity fibular pseudarthrosis with latent pseudarthrosis of tibia progressive valgus deformity of ankle develops. A 4yearold girl with neurofibromatosis type 1 nf1 was admitted to the emergency department, with a leftsided limp and pain. Pseudarthrosis pain in the spine pseudarthrosis treatment. Management of posttraumatic pseudarthrosis of tibia with. The condition exhibits a wide range of severity and the response to treatment is unpredictable. Free vascular fibular transfer in congenital pseudoarthrosis of the tibia.
Pdf electrical stimulation in the treatment of congenital. Introduction congenital pseudarthrosis of tibia refers to nonunion of tibial fracture that develops spontaneously or after trival trauma in a dysplastic bone segment of tibia diaphysis. Several surgical methods are available for its treatment1. Pseudarthrosis, or pseudoarthrosis, implies the formation of a false joint, usually following a fracture, with no attempt at repair. At the site of insufficient bone healing, white fibrous tissue substitutes the bone. Congenital pseudoarthrosis in a child with neurofibromatosis. The condition is usually apparent shortly after birth and is rarely diagnosed after the age of two. Tibial pseudarthrosis occurs in association with neurofibromatosis in. Thabet am, paley d, kocaoglu m, eralp l, herzenberg je, ergin on. The fibular pseudarthrosis is approached by dissecting posterolateral to the tibia. Feldman presenting live on congenital pseudarthrosis. Free vascularized fibular graft for tibial pseudarthrosis in neurofibromatosis. A pseudarthrosis is defined as a false joint and is a break in the bone that fails to heal on its own. The patient can have full weight bearing and be free of pain.
Complex radiological findings in congenital pseudarthrosis. There is fluid and a joint like appearance to this space. A retrospective study will be conducted with around 150 patients at 11 centers in china. Campanacci and zanoli 5 described a fibula pro tibia technique with fibular 18 fixation to the pseudarthrosis site. What does a spine pseudarthrosis pseudoarthrosis look like. The purpose of this study was to access the surgical results, complications, secondary procedures, and long. Management of congenital pseudoarthrosis of the tibia and. Congenital pseudoarthrosis of the tibia describes abnormal bowing that can progress to a segment of bone loss simulating the appearance of a joint. Posttraumatic pseudarthrosis nonunion of tibia is a challenging problem in orthopaedic practice. It has become accepted that this lesion is associated with an. All five transfers have united and hypertrophied with growth. Congenital pseudarthrosis of the tibia cpt is one of the most challenging problems in paediatric orthopaedics. Congenital pseudarthrosis of the tibia paley orthopedic.
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