Pediatric Spine Foundation

Title: Growth-Friendly Spine Surgery in Arthrogryposis Multiplex Congenita (AMC)

Authors: Bram P. Verhofste, MD, John B. Emans, MD, Patricia E. Miller, MS, Craig M. Birch, MD, George H. Thompson, MD, Amer F. Samdani, MD, Francisco J. Sanchez Perez-Grueso, MD, Anna M. McClung, BSN, Michael P. Glotzbecker, MD, Pediatric Spine Study Group

Journal: The Journal of Bone and Joint Surgery

Date: January 20, 2021

Excerpt: Our study objectives were to describe current growth friendly instrumentation (GFI) trends in children with AMC and early-onset scoliosis (EOS) and to compare long-term outcomes with a matched idiopathic EOS (IEOS) cohort to determine whether spinal rigidity or extremity contractures influenced outcomes.

Keywords: Arthrogryposis Multiplex Congenita, Growth-friendly, Growth-friendly Spine Surgery, GFI

Summary:

Purpose

: Arthrogryposis Multiplex Congenita (AMC) is characterized by multiple joint contractures, permanent bends or fixtures in the joint, at birth. This study aims to describe current trends in Growth Friendly treatment in children with AMC and early-onset scoliosis (EOS) and to compare long-term outcomes with a matched non-AMC patient to determine whether AMC affects treatment results and quality of life.

Results

  • Patients with AMC had larger initial coronal curves (55°) and less improvement in coronal correction post procedure (5°) in comparison to the matched non-AMC patient (43° and 8°).
  • Patients with AMC developed 51% more complications than matched non-AMC patients.
  • Both groups were given the EOSQ-24, a questionnaire covering topics including quality of life, pain, and family burden. The mean total scores improved after treatment for both groups. There were no significant differences in the general health, pain, pulmonary, or emotion subdomains. The quality-of life subgroup scores were consistently lower in the AMC group.

Discussion Points

  • Arthrogryposis Multiplex Congenita patients had an increased risk of complications than non-AMC patients. Scores of quality-of-life were also lower in AMC patients.
  • Patients with AMC and EOS experienced less initial correction after the procedure, but final curve magnitudes during treatment were comparable to patients without AMC. Growth-friendly treatment is successful in correcting EOS in patients with AMC, however complication rates and other risks are an important consideration when discussing treatment options.