Congenital anomalies of the lower extremities – the extensive and various group of malformations including pathology of area of a coxofemoral joint, hip, knee joint, shin, ankle joint and foot. Total absence of an extremity or some of its departments, an underdevelopment of the whole segment or one of the bones entering this segment (for example, only tibial or only low-tibial), an underdevelopment of muscles, vessels and nerves, soyedinitelnotkanny banners etc. can be observed. The combination of several congenital anomalies is possible. Influence of adverse external and internal factors during pregnancy is the cause of malformations. Hereditary predisposition is in certain cases observed. The diagnosis is exposed on the basis of yielded survey, results of a X-ray analysis, KT, MPT and other researches. Treatment is usually surgical. The forecast depends on weight of pathology.
Congenital anomalies of the lower extremities
Malformations of the lower extremities represent extensive group of the anomalies which arose in the pre-natal period and considerably differing on weight. In practice of orthopedics and traumatology meet rather often and make 55% of total of congenital anomalies of the musculoskeletal device. Rough defects are diagnosed right after the birth. Small anomalies in some cases can proceed asymptomatically or almost asymptomatically and to become a casual find at inspection concerning other injuries and diseases. Children's orthopedists and traumatologists are engaged in treatment of congenital anomalies of the lower extremities.
Anomalies of development can result from mutations, and also action of external and internal teratogenny factors. The most widespread external factors exerting negative impact on formation of extremities are infectious diseases, violations of food, reception of a number of medicines and radiative effects. Advanced age of mother, uterus pathology, serious somatic illnesses, some gynecologic diseases and endocrine violations belongs to number of internal factors which can lead to violation of formation of extremities.
1. Defects which arise owing to insufficiency of formation of any parts of the lower extremities
Amelia – an extremity completely is absent. The opus (both lower extremities are absent) and is possible (one lower extremity is absent).
Fokomeliya or tyuleneobrazny extremity. There are no middle and/or proximal parts of an extremity together with the corresponding joints. Can be bilateral or unilateral. Sometimes all extremities, both lower, and top are involved in process. A full fokomeliya – the shin and a hip are absent, the created foot is attached to a trunk. The disteel fokomeliya – a shin is absent, foot is attached to a hip. The proximal fokomeliya – a hip is absent, a shin with foot are attached to a trunk.
Peromeliya – a kind of a fokomeliya at which lack of a part of an extremity in combination with an underdevelopment of its disteel department (foot) is observed. The full peromeliya – a leg is absent, in the place of its attachment the skin ledge or a rudimentary finger settles down. Incomplete – the hip is absent or is underdeveloped, the extremity also comes to an end with a skin ledge or a rudimentary finger.
Besides, distinguish lack (aplaziya) of a low-tibial or tibial bone, lack of phalanxes (afalangiya), lack of fingers (adaktiliya), existence of one finger on foot (monodaktiliya), and also typical and atypical forms of splitting of foot because of absence or an underdevelopment of its average departments.
2. Defects which arise owing to an insufficient differentiation of any parts of the lower extremities
Sirenomeliya – merge of the lower extremities. Merge only of soft fabrics or merge of both soft fabrics, and some tubular bones can be observed. It is quite often combined with absence or an underdevelopment of bones of a basin and extremities. At a sirenomeliya both lack of feet, and existence of one or two feet is possible (is more often than rudimentary). The simultaneous underdevelopment of a rectum, back pass, uric system, internal and genitalia is usually observed.
3. Defects owing to increase in quantity: increase in number of the lower extremities – a polimeliya, doubling of foot – a diplopodiya.
4. Defects owing to insufficient growth – hypoplasias of various bones of the lower extremities.
5. Defects owing to excess growth – the giantism arising at unilateral increase in the developed extremity.
6. Congenital banners – the fabric tyazh arising in various places of an extremity and often breaking its function.
Makes 1,2% of total of congenital deformations of a skeleton. It is often combined with other anomalies, including an aplaziy low-tibial bone and lack of a patella. Are shown by lameness. Extent of dysfunction of an extremity depends on the size of shortening and weight of a malformation. At defeat of a diaphysis adjacent joints are, as a rule, not changed, their function is kept in full. At defeat of disteel departments of a hip usually there are contractures. The extremity of a rotirovan, is shortened. Basin is warped and lowered towards defect. The buttock fold is maleficiated or is absent. Muscles of a buttock and a hip are atrophied, foot in the provision of an ekvinus. The X-ray analysis of a femur demonstrates shortening and an underdevelopment of a segment.
Treatment is surgical, directed to restoration of length of an extremity. At early age carry out operations for stimulation of rostkovy zones. Since 4-5 years, carry out an osteotomiya in combination with imposing of the distraktsionny device. If shortening is so big that restoration of length of an extremity is not possible, amputation of foot, sometimes – in combination with artrodezy a knee joint is necessary (for creation of a long functional stump). At insignificant shortening use of special footwear and various orthopedic devices is possible.
Congenital dislocation of a hip and dysplasia of a coxofemoral joint
Congenital dislocation of a hip is observed rather seldom, various degrees of a dysplasia usually are found. Pathology, as a rule, unilateral. Girls suffer 7 times more often than boys. In 5% of cases direct transfer of defect by inheritance comes to light. It is shown by lameness, rotation and shortening of an extremity. At bilateral anomaly there is a duck gait. The X-ray analysis of a coxofemoral joint demonstrates reduction and flattening of a head and its standing above a vertluzhny hollow. Treatment at early age conservative with use of various devices, special shorts and small pillows. At ineradicable dislocations on reaching 2-3 years operation is performed.
Valgusny and varusny deformation of a hip
Develops at violation of an ossifikation of a neck or pre-natal injury of a cartilage, it is equally often observed at girls and boys, in 30% comes to light from two parties. Valgusny deformation, as a rule, proceeds asymptomatically. The Varusny curvature is followed by lameness, restriction of movements and bystry fatigue of an extremity. Clinical manifestations remind congenital dislocation of a hip. At a X-ray analysis the delay of ossification of a head, shortening and thinning of a femur is defined. Cervical the corner is reduced. Treatment is surgical, the corrective osteotomiya for increase cervical a corner is carried out.
Congenital dislocation of a patella
Meets rather seldom. There is a hereditary predisposition. It can be combined with other defects, boys suffer twice more often than girls. Congenital dislocation of a patella is shown by bystry fatigue of an extremity, unstable gait and frequent falling. The contracture is possible. Without treatment the problem is aggravated with age, there is a deforming arthrosis, the valgusny curvature of an extremity develops. The X-ray analysis of a knee joint testifies to an underdevelopment and shift of a patella (knaruzh are more often) and an underdevelopment of an external condyle. Treatment surgical – own ligament of a patella is moved and fixed in median situation.
Lack of a patella
It is often combined with other anomalies of development of a knee joint (an underdevelopment of the articulate ends tibial and a femur), with dislocation of a hip and shin, a clubfoot and other defects. The course of the isolated pathology usually asymptomatic, at the raised loadings weakness and fatigue of an extremity is possible. At the isolated anomaly treatment is not required.
Congenital dislocation of a shin
Comes to light seldom, usually has bilateral character. Is followed by a contracture and deformation of a knee. The type of deformation depends on a type of shift of bones of a shin. Muscles of a hip and a shin are underdeveloped, often have abnormal points of an attachment. Pathology is quite often combined with anomalies of development of an ankle joint, absence or an underdevelopment of a tibial bone. Treatment at early age conservative (extension with the subsequent reposition). At the age of 2 years is also more senior operations – open reposition of dislocation, in need of a combination to correction of the accompanying skeletal pathology are performed.
Valgusny and varusny deformations of a knee joint
Are observed seldom, can be descended. Are usually combined with deformation of a neck of a hip and flat-footedness. Become the reason of an early heavy gonartroz. Aged up to 5-6 years correction with use of conservative methods is carried out, in the subsequent surgery is performed. Depending on weight of pathology carry out the isolated osteotomiya in the field of nadmyshchelok of a femur or combine a hip osteotomiya from a zhelobkovy, wedge-shaped or cross osteotomiy tibial bone.
Aplaziya or underdevelopment of a tibial bone
Is followed by shortening and a curvature of an extremity. Foot of a supinirovan, is in the provision of an ekvinus or incomplete dislocation. The support is broken. The combination to an underdevelopment or lack of bones of foot, an underdevelopment or dislocation of a patella, an atrophy and violation of development of muscles of a shin and hip is possible. To children up to 3 years conservative therapy for recovery of the normal provision of foot is carried out. In the subsequent lengthening of a shin with use of distraktsionny devices is carried out.
False joint of a tibial bone
Can be true or arisen in the location of a congenital cyst. Pathological mobility, an ugloobrazny or arc-shaped curvature in a false joint, an atrophy of muscles, consolidation and cicatricial changes of skin, shortening and thinning of an extremity comes to light. The X-ray analysis of bones of a shin testifies to osteoporosis. Treatment surgical with use of bone transplants or Ilizarov's device.