Prosthetics and Orthotics International

This classification originally produced by the ISPO “Kay” Committee in 1973, has now with minor modifications, become an International Standard (ISO 8548-1: 1989). It is limited to those deficiencies which are failures of formation and describes them on anatomical and radiological bases only. All are divided into transverse and longitudinal, and use simple terms and descriptors.

Isolated limb deficiencies are usually sporadic occurrences. However, if they are associated with other abnormalities or a family history, the risk to future pregnancies may be as high as 50%. A thorough history, examination and investigation of the baby as well as the parents is essential before assessing this recurrence risk. The syndromes associated with limb deficiencies are presented.

A properly constituted clinic team needs to be involved as soon as possible after the birth of a child with a limb deficiency, to answer the parents' questions, counsel them and the family, and plan the treatment programme for the child so that they can properly assist the child to become a productive, self-sufficient member of society.

The provision of prostheses for children with upper limb deficiency starts at an early age. The roles of the occupational therapist and prosthetist in this programme are described. Casting and fitting techniques applicable to transverse forearm loss and aspects of myoelectric hand prostheses are included. An outline of training methods as related to both parent and child is explained. Higher levels of limb deficiency are covered briefly and the use of alternative methods of electric hand control is included. Finally, a brief summary of distal deficiency is discussed.

This paper presents an occupational therapy method for training children fitted with myoelectric prostheses. It is based upon a structured way of describing the accomplishments of a child fitted with a myoelectric prosthesis, called the Skill Index Ranking Scale (SIRS).

By using the SIRS when designing the training session, the therapist can progressively increase the demands presented to the child. Furthermore, the SIRS facilitates for the therapist the documentation and communication of the childs ability with the myoelectric prosthesis.

For those with levels of loss above the elbow, the normal prescription choice is now considered to be the use of an electric elbow combined with a myoelectric hand using a single site three state control. The lightweight VASI unit controlled by a harness mounted switch is mounted in a prosthesis the socket of which is total contact, and allows improved power transfer.

Terminal overgrowth in transverse deficiencies and in amputations, particularly of the humerus and tibia necessitate serial trimming procedures or re-amputation. Capping using an autogenous bone graft or a titanium and polyethylene endoprosthetic cap provides a satisfactory way of voiding these re-amputations and allowing end-bearing.

Absence of the radius is the commonest of the longitudinal deficiencies but is itself quite an ususual congenital anomaly. No one surgeon is likely to encounter the condition more than once or twice in a career and there is a strong case for congenital upper limb conditions to be collected into special clinics.

The pathology and methods of surgical treatment are reviewed. It is a difficult condition to treat but there is no doubt that function and appearance can be improved (Pulvertaft, 1973). Pollicisation of the index finger should be considered and can often greatly improve the function.

Longitudinal ulnar deficiency, whose detailed anatomy is varied, is often accompanied by other abnormalities and appears as part of several syndromes. The history of its classification is reviewed, and the treatments, which have been offered, described.

Follow-up of a series shows that the function achieved is good and is optimised by aids, occupational therapy and some hand surgical procedures. The more elaborate surgical reconstructions are unlikely to be beneficial.

This review describes the two types of congenital cleft hand deformity, stressing the important ways in which they differ and summarises the surgical techniques that may be used in their treatment.

The prosthesis required for a child with a transverse deficiency whilst a simple version of that appropriate for the adult follows the same principles. The child with a longitudinal deficiency may require an extension, or orthoprosthesis and this may be combined with surgical reconstruction. The principles, technique of measurement and fabrication methods using both traditional and modern composites are described.

After a review of previous classifications the authors suggest that cases of congenital longitudinal deficiency of the femur fall into one of two groups, each with specific features. The treatment alternatives and their indications are discussed in detail and a treatment plan suggested.

Partial tibial absence and congenital ankle diastasis are described together with a surgical regime for their reconstruction.

Recent literature on the subject is reviewed, and the role of Syme's amputation, reconstructive surgery and prosthetic management is discussed in relation to the severity of the condition. Amputation, which should be performed between 18 months and two years old is specifically recommended for total fibula absence with ankle instability. The operative technique is detailed.

Three types of fibular deficiency are described which determine the nature of the surgery and prosthesis required. The surgical management of 50 patients who had a total of 103 operations is described.

The incidence of limb deficiency is given as is the prevalence of prosthetic use in Australia. The organisation of clinics and the Free Limb Scheme is described and its effects discussed.

The problem of treating limb deficient children in India is compounded by many factors, social, cultural and economic. Few attend early and most therefore manage without prosthetic care.

The incidence as well as the current state of the management of the limb deficient child in Japan is described.

The surgical and prosthetic treatment of longitudinal lower limb deficiency is described and discussed, in the light of cultural and social requirements. Those with upper limb deficiencies are not fitted with prostheses.

An overview of the situation in Venezuela is made by sampling the cases of congenital limb deficiency treated at the Hospital San Juan de Dios in Caracas from 1961-1989. The major longitudinal deficiencies are analysed.

The socioeconomic situation of the patients is of most importance being the cause of final decisions in relation to surgery, the ordering of prosthesis and orthosis, supplied mainly by the Venezuelan Institute of Social Security (IVSS), the maintenance of these items and the possibility of travelling to the hospital for diagnosis, treatment and follow-up.

All these transform the situation from a medical problem into a socio-economic problem typical of a third world country.

The use of a parent support organisation and its responsibilities and limitations are described. The difference between a parent support organisation and an association of limb deficient children is discussed.

David Bailey, who is 14 years old, has a left transverse total forearm deficiency and bilateral longitudinal femur partial deficiencies. The latter fall into Torode and Gillespie's Group I, with marked femoral shortening. A wedge osteotomy was performed for his gross right coxa vara, which was successful, but an intractable fixed flexion deformity of the right knee led eventually to a knee disarticulation being performed.