Short-term functional outcome in children with arthrogryposis multiplex congenita after multiple surgeries at an early age

Figures & data

Table 1 Clinical profile of children with arthrogryposis multiplex congenita

Order of patient	Sex	Age at last follow-up	Duration of follow-up	Hip	Knee	Foot	Shoulder	Elbow	Hand and wrist	Spine
1	M	6	4	Bilateral dislocation	Bilateral extension			Bilateral flexion contracture
2	F	4	2.5	Bilateral dislocation	Bilateral extension
3	M	6	4	Bilateral dislocation	Bilateral dislocation	Bilateral equinovarus	Bilateral adduction	Bilateral flexion contracture	Bilateral extension contracture
4	M	4	3	Rt: dislocation	Rt: flexion contracture, Lt: extension contracture	Bilateral equinovarus			Bilateral flexion contracture with ulnar deviation
5	M	4	3	Rt: dislocation		Rt: equinovarus		Bilateral flexion contracture	Bilateral flexion contracture, bilateral clasped thumbs	Scoliosis
6	M	5	4	Bilateral flexion contracture		Bilateral equinovarus
7	M	2.5	2		Bilateral dislocation			Bilateral flexion contracture
8	M	3	2.5	Bilateral abduction and external rotation contracture	Bilateral flexion contracture	Bilateral equinovarus
9	F	2.5	1.5	Bilateral flexion contracture	Bilateral flexion contracture
10	M	3	2	Bilateral dislocation		Rt: equinovarus
11	M	6	5		Bilateral flexion contracture	Bilateral calcaneovalgus
12	F	13	6	Bilateral dislocation	Bilateral flexion contracture			Bilateral flexion contracture	Bilateral flexion contracture
13	M	8	5	Bilateral dislocation	Bilateral extension	Bilateral equinovarus	Bilateral adduction contracture		Bilateral flexion contracture
14	M	15	6	Bilateral flexion contracture	Bilateral extension	Bilateral equinovarus		Bilateral flexion contracture	Bilateral flexion contracture	Kyphoscoliosis

Table 2 Surgical procedures performed

Type of surgery	Number	Range of age (months)	Mean of age (months)
Hip
Open reduction (primary)	13	8–28	18.5
Open reduction (revision)	2	12–16	14
Pelvic osteotomy	11	16–28	20.8
Derotation varus osteotomy of femur with shortening	5	16–24	18.2
Open adductor tenotomy	8	9–28	18.1
Knee
Posterior soft tissue release (primary)	9	8–24	17.9
Posterior soft tissue release (revision)	4	24–32	28.5
Distal femoral extension osteotomy	4	26–42	33.5
Quadriceps plasty	9	4–28	19.6
Foot
Complete subtalar release (primary)	14	8–32	20.6
Complete subtalar release (revision)	2	26–66	36
Upper limb
Posterior elbow release and triceps plasty	1		28
Carpal osteotomy	1		36

Figure 1 Clinical picture of the child who was nonambulatory at last follow-up, showing severe involvement of the upper and lower limbs at birth.

Table 3 Functional outcomes for children with arthrogryposis multiplex congenita

Functional status	Number of patients	Mean age (years)	Activities of daily living
Independent walker	6	8.3	Not affected
Walks with support	3	3.5	Not affected
Household walker	1	3	Not affected
Nonfunctional ambulator	3	4.2	Not affected (two patients) Limitation in one patient
Nonambulator	1	5	Severe limitation