Kongress ORTHOPÄDIE + REHA-TECHNIK 2012Poster [4311] Abstract [975] | Thema: Rehabilitation

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AuthorKoltsov, Andrey (Saint-Petersburg RU)Saint-Petersburg Albrecht Center for Occupational Expertise, Prosthetics and Rehabilitation -First orthopedic departement for childs

TitleReconstructive surgery and prosthetic in rehabilitation of children with proximal ectromelia ofupper extremities

Coauthors

I. Schvedovchenko

Summary

The results of rehabilitation of children with proximal upper-extremity ectromelia are presented.

We used reconstructive surgery and microsurgery for restoration of anatomy and function of

upper limbs, simetimes in combination with prostheses.

Introduction

Proximal upper-extremity ectromelia is one of rare and severe forms of congenital pathology

which is characterized by hypoplasia of all limb segments with primary affection of proximal

parts.

In the literature we have found some other definitions of this anomaly, for example «axial

ectromelia» (L. Henkel, HG. Willert, 1969) and «phocomelia» (C.H. Frantz, R. O´Rahilly, 1961;

A.B. Swanson et al., 1983; G. Tytherleigh – Strong, G. Hooper, 2003). Latter definition is most

widely used, because quite often the underdeveloped extremity looks like seal or walrus fin.

Clinical signs of the anomaly are clavicle and scapula hypoplasia, shortening of all parts of

the extremities. The shoulder joint is always absent, humeral bone, elbow and wrist joints

are hypoplastic or are absent. In most cases fingers have 3 phalanges, and the thumb is

underdeveloped or is absent.

Methods

We have treated 62 patients, from 1 month old up to 46 years old, total number of extremities

82. 30 patients had left-hand, 12 patients - right-hand, 20 persons - bilateral defect.

The patients were divided into 3 groups:

Kongress ORTHOPÄDIE + REHA-TECHNIK 2012Poster [4311] Abstract [975] | Thema: Rehabilitation

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31 patient with typically bilateral defect, with presence of all 3 segments of the extremity -

rudimentary distal part of humeral bone and sometimes hypoplastic elbow joint, forearm and

hand. Most patients were actively using the underdeveloped extremity for self-care. All patients

used bimanual grasp.

24 patients, the affected extremities were only hypoplastic bones of forearm or uncertain bone

formations between the underdeveloped hand and scapula. The capability depended on the

forearm bones hypoplasia degree.

10 patients, the underdeveloped extremity was a faulty hand, adjacent to the scapula. Such

patients had highest functional impairments, they can only to keep the objects between

rudimentary extremity and body.

Results

In group 1 surgical treatment was the most frequent, while the prosthetics – the most rare.

More, then 25% patients of group 1 did not get some surgical unterventions or prostheses.

In opposite, the frequency of surgical treatment in group 2 and 3 was lesser, and frequency of

prosthetics – higher, espacially in group 3. All patients of group 2 and 3 got surgical treatment

or prosthetics.

Surgical treatment was multistage. 37 operations in 13 patients, age from 11 months up to

15 years, in most cases the surgical treatment was carried out in patients of group 1A. Most

operations were independent method of treatment and only 3 operations were made as a

preparation to the subsequent prosthetics.

For formation of thumb and creation of an opportunity of bilateral grasp we have carried out

pollicization,

reconstruction of radial finger, microsurgical autografting of second toe to the position of the

thumb.

The hand operations in all patients have resulted in formation of opposed thumb, which

provides an opportunity of bilateral grasp.

In order to create a humeral bone we have carried out transposition of external edge of

corresponding scapula with vascular pedicle to the position of severely underdeveloped

humeral bone. In order to create a correct shape and position of the shoulder girdle we have

Kongress ORTHOPÄDIE + REHA-TECHNIK 2012Poster [4311] Abstract [975] | Thema: Rehabilitation

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carried out shoulder girdle reconstruction as a preparatory stage for prosthetics. This operation

has helped to simplify the process of prosthetics fitting.

Conclusion

1. Reconstructive surgery and prosthetics can improve function and appearance of

underdeveloped upper extremities in patients with severe congenital anomaly - proximal

ectromelia, espacially with presence of rudimentary humeral bone and hypoplastic elbow joint.

2. Basic methods of surgical treatment are the methods aimed at formation of thumb and

bilateral grasp, reconstruction of humeral bone and shoulder girdle.

3. Surgical treatment is multistage and should be started as early as possible.

4. Surgical treatment usually is independent, but sometimes can be as preparatory stage for the

subsequent prosthetics.

References

Shvedovchenko I. Surgical treatment of of proximal upper-extremity ectromelia in children / I.

Shvedovchenko, V. Prokopovich // The traumatology and orthopedics Herald by N.N. Priorov. –

2002. – N 4. - P. 73 – 76.

Shvedovchenko I. The treatment of children with congenital anomalies of upper limbs / I.

Shvedovchenko // Traumatology and Orthopedics – St.-Petersburg: Hippocrate, 2005. – Vol. 2.

- P. 634 – 664.

Shvedovchenko I. The method of correction of shoulder girdle deformation / I. Shvedovchenko,

A. Koltsov, A. Korukov // – Russian Federation patent for invention N 23321382 from

28.12.2006.

Agranovich #. The method of forming of the hand´s grasp / O. Agranovich, I. Shvedovchenko,

A. Koltsov // – Russian Federation patent for invention N 2339330 from 10.01.2007.

Koltsov A. Clinical and roentgen features and classification of proximal upper-extremity

ectromelia (revue) / A. Koltsov // The traumatology and orthopedics of Russia. – 2007. – N 3. –

P. 87-94.

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