TREATMENT OF BILATERAL ELBOW EXTENSION CONTRACTURE IN ARTHROGRYPOSIS
Christina N. Yu Bsc, OT; Michael Lenz, CP; Judy Thomson, RN BN; Mei Fong, FT; James Harder, BScMD, FR(CO)
The name arthrogryposis is a direct translation from two Greek words: arthron and gryposis. "Arthron" means joints and "Gryposis" means bending.
Different types of arthrogryposis can be distinguished based on determining whether there is (type I) primarily involvement of just the limbs, (type II) limb involvement with other abnormalities, or type (111) involvement with major central nervous system dysfunction.
The Type I arthrogryposis, often referred to just arthrogryposis, is also known as Amyoplasia which is characterized by a very specific symmetric positioning and fibrotic replacement of muscles. It most commonly involves all 4 limbs, or in some cases, only the lower extremities are involved. It is less common to have only the upper extremity involvement.
In the upper extremities, the classical type I arthrogryposis is characterized by internal rotation and adduction of the shoulders, rigidly flexed or extended elbows, and pronated forearms at birth. There are flexed and ulnarly deviated wrists, with very little muscle mass of the arms. Fingers are flexed and thumbs adducted.
In the lower extremities, the feet are always in severe equinovarus positon. The knees may be flexed or extended. Hips may be in almost any position but are usually involved and frequently dislocated.
Treatment for this population usually involves early casting, splinting, and stretching of the involved limbs. Functional treatment to promote independence in activities of daily living is essential. Surgical intervention is often involved in the lower extremities. In the upper extremities, treatment is more controversial. The lengthening of the triceps tendon and posterior capsulotomy and the triceps transfer has been performed to obtain passive elbow flexion in some patients.
A general upper extremity functional goal is to enable one extremity to be flexed and brought to the face for feeding and hygiene and one to be extended to be used to push up from a sifting position, for toileting, or to be used with a crutch if needed.
For the arthrogryposis population, since some of the patients may utilize their contractures and deformities to perform certain tasks, such as using two pronated forearms and flexed wrists in a scissored fashion for grasp, it is important to determine what functions the patient can perform and in what way, so that when considering any treatment, any existing function will not be taken away without improving it.
At the Alberta Children's Hospital, arthrogryposis children are seen under the Juvenile Amputee Clinic which is a multidisciplinary team consisting of a pediatric orthopaedic surgeon as team leader, a nurse as a clinic coordinator, an occupational therapist, a physical therapist, and a group of prosthetists from the local and the surrounding areas.
PATIENT OF DISCUSSION ALLISON
Allison is a 4 years 5 months old girl born with arthrogryposis. Both of her upper extremities are involved. Her lower extremities are normal. The team has been involved since infancy.
At birth, Allison presented with adducted and internally rotated shoulders with very little girdle muscle noted. Elbows were in extension. The hands and wrists were clublike, i.e., the wrists were contracted in flexion with slight ulnar deviation. The thumbs were adducted and flexed in a palmar direction. Very small amount of active shoulder movement in an anterior direction was noted. No active or passive elbow movements on the left were noted. About 40 degrees passive elbow flexion was noted on the right during infancy which was, later, increased to 80 degrees with stretching and splinting.
Allison suffered a supracondylar fracture of the left humerus in October 1999 and again fractured her right olecranon in December 1999. Her right elbow was casted in 90 degrees flexion. This degree of flexion has been maintained after the cast was removed by stretching and splinting. This resulted in an increase in the passive elbow flexion on the right side.
Allison can perform different tasks in a few different positions.
In sitting, Allison uses her knees to guide the movements of her hands. She can perform tasks such as beading, building with I - blocks, and drawing/writing in this position.
When sitting on a chair, Allison can put her right leg up on the table, using the knee to guide the movement of her right hand. She usually uses this position to feed herself, especially out in a restaurant.
When standing, Allison swings her right arm (the one with about 90 degrees passive elbow range) up on a surface and get the hand close to her face by flexing the elbow passively and leaning her body forward. She can perform activities such as brushing her teeth, cleaning her face and feeding in this position.
Allison is now 4 years 5 months old. She lives with her parents and her younger brother. She goes to a special educational preschool 5 half days.
Feeding: Allison can use a spoon or fork with both of her hands together. She prefers to finger feed herself with her right hand. She can drink with a straw or use her teeth to bite at the rim of a cup and bring the cup up to her mouth to drink.
Dressing: Allison can pull shoes and socks off with her feet but can not put them on. She needs help for fasteners and getting shirts on and off. Allison has difficulties pulling pants on or off past mid-thigh level. O.T. has put hooks on the wall at about knee level and put loops on pants to assist in pulling pants up and down. After some practice, Allison can now get pants on and off independently.
Toileting: Presently, Allison requires assistance for wiping. O.T. is working on increasing independence in this area.
Writing/drawing: Allison is capable of drawing on paper, but strokes and pressure are very weak. Grasp is mainly between the II and III fingers on the left side.
Playing: Allison prefers to sit on the floor to play. With a weak grasp, Allison mainly plays with lightweight toys such as beads, and drawing with markers.
Grasp is generally weak but adequate in performing activities such as putting beads in a bottle. Allison can carry a light object with two hands or between her upper arm and the side of her body, which is sometimes called the bra-chial-thoracic grasp.
For Allison, a stretching and splinting program was initiated in infancy. Some increase in passive wrist extension has been achieved bilaterally. The right elbow has also showed some increase in passive flexion from about 40* to about 80* before the fractures. The passive elbow flexion of the right side has increased to 90* after the fracture and the casting. There was no significant change in her left elbow. Promoting independence in functions has been ongoing.
The option of feet use has been explored with parents since infancy. Recently, Allison and her parents become more interested in the use of her feet for some activities, such as using the computer adapted and \ manipulating the track ball.
ONGOING REHABILITATION PROGRAM
Continuous efforts have been focused on improving the efficiency of grasp patterns, exploring and designing various devices, such as hooks on walls and altered clothings to increase independence in dressing and undressing, and toileting.
Although Allison's functions have been limited by the extension contractures and the lack of active movements, Allison has been able to utilize the limited passive elbow flexion on the right side to perform some major functional tasks, such as feeding. She is capable of swinging her right arm up onto a surface and leaning her upper body forward to her hand to utilize the hand function she has.
A joint attempt by the O.T. and the prosthe-tist, Mike Lenz, was made to determine if the passive elbow range on the right side can be maintained, and therefore, can be further utilized to increase function. An elbow splint with a ratchet hinge that locked elbow in various degree of flexion was fabricated.
The hinged splint has 4 positions of flexion available by pushing against a surface to lock the splint in a flexed position. More bending and pushing will lock the splint in an increased flexion position. Bending the splint to the end position would release the lock and allow it to go back to extension.
Age: This ratchet-hinged splint was first initiated in March 1999 to experiment with different kinds of orthoses to determine if the existing passive elbow flexion can be further utilized to increase functional level.
Allison did not wear the splint consistently. She was only 3 years of age when the splint was initiated. It was challenging to demand her to wear the splint on a consistent basis.
Single axis hinge: It was also noticed that Allison's right elbow goes into slight pronation while flexing. Since the splint only allows single axis flexion /extension, when Allison's elbow goes into flexion, the splint pinches and twists her skin and seems to be uncomfortable to wear.
Not sensible to range adjustment needs : the splint locks the elbow in a certain angle of flexion. To change into another position, the person has to unlock and then lock at a different degree of flexion again. Allison needs to constantly adjust her elbow flexion for activities. This need for range adjustment, however, cannot be matched by the splint.
Functional with existing range and strength: Allison is very functional using her arms in her daily activities. Also, it is important to consider the fact that she has normal lower extremities. She has already started to learn to perform certain tasks with her feet such as using the computer and picking up objects with her feet. Allison may not be, therefore, as motivated to try using the splint since she has other options.