Orthopaedic care of the child with arthrogryposis: a 2020 overview

Abstract

Purpose of review The orthopaedic treatment of children with arthrogryposis multiplex congenita has evolved steadily over the past two decades. Interrelated factors have spurred this on, including better appreciation of the functional potential of persons with arthrogryposis, development of newer procedures specific for the arthrogrypotic deformities, and outcomes studies that provide understanding of the overall capabilities of adults with arthrogryposis and follow-up to determine which treatments were beneficial and which were not. This article briefly sketches out of some of these advances and indicates areas that need further development. Recent findings Outcome studies show that the majority of adults with arthrogryposis are ambulatory but less than half are fully independent. Adults frequently experience ongoing pain, particularly foot and back pain, limiting ambulation and standing. Advancements in the upper extremity treatment include improving elbow function, wrist repositioning, and improving thumb positioning. In the lower extremities, correction of hip and knee contractures leads to improved ambulatory potential, and treating clubfeet with serial casting decreases poor outcomes. Summary Clinical evaluation, both physical examination and assessment of the patient's needs, are important in directing treatment in arthrogryposis. Further outcomes studies are needed to continue to refine procedures and define the appropriate candidates.

Surgical Treatment of Children with Hip Dislocation in Amyoplasia-Type Arthrogryposis: A Rational Approach to Treatment Selection

“…Анализ литературы по данной проблеме за последние 10 лет показал, что большинство публикаций представлены небольшими сериями клинических случаев, посвященных раннему хирургическому лечению, а именно выполнению операции открытого вправления бедра (ОВБ) в сочетании с периартикулярными релизами. При этом хорошие результаты лечения варьируют в широком диапазоне -от 54 до 90% случаев, однако имеются сообщения, указывающие на неэффективность данного вмешательства [10,11,12,13]. Некоторые авторы предлагают дополнять ОВБ корригирующей остеотомией бедренной кости трАВмАтОЛОгИЯ И ОртОпЕДИЯ рОССИИ / TrAUmAToLogy AND orThopEDICS of rUSSIA (КОБ) и различными вариантами периацетабулярных остеотомий или ацетабулопластик в более старшем возрасте, но при этом акцентируют внимание на наибольшем количестве послеоперационных осложнений у детей старше 3 лет.…”

Total joint replacement of the hip and knee in patients with arthrogryposis multiplex congenita: a report of six joints

“…Arthrogryposis multiplex congenita (AMC) is a rare congenital symptom complex that describes a collection of aetiologies with several significant joint contractures and consequent deformities [1][2][3]. While the condition is uncommon, a high percentage of patients have relevant pathologies of the lower extremities, particularly of the hip and knee joints [2,3]. Many arthrogryposis hips are dislocated or demonstrate severe flexion contractures which require surgical reduction or release during childhood and adolescence [1,2,4,5].…”

“…While the condition is uncommon, a high percentage of patients have relevant pathologies of the lower extremities, particularly of the hip and knee joints [ 2 , 3 ]. Many arthrogryposis hips are dislocated or demonstrate severe flexion contractures which require surgical reduction or release during childhood and adolescence [ 1 , 2 , 4 , 5 ]. In more severe cases or recurrent dislocation, corrective osteotomies may be required [ 4 ].…”

“…In more severe cases or recurrent dislocation, corrective osteotomies may be required [ 4 ]. The knee joints commonly display flexion or extension contractures which can be corrected by soft tissue release or extension osteotomies during childhood and adolescence [ 1 , 2 ].…”

“…Considering that a high percentage of patients with AMC has the potential to ambulate in adulthood and has a close to normal life expectancy [ 6 , 7 ], surgical intervention to correct deformities or to release contractures should be performed to maintain or regain long-term ability to ambulate and to prevent or relieve pain [ 2 , 7 ]. However, due to the often severe and recurring contractures and deformities [ 1 , 5 , 8 ] some patients may develop degenerative joint changes that cause pain and limited function [ 9 ].…”