1131 - Pediatric Post-Surgical Rehabilitation in a Patient with MYH3-Associated Arthrogryposis Multiplex Congenita: A Case Report

A 9-year-old female with a history of MYH3 mutation, arthrogryposis, scoliosis, and popliteal pterygium presented to inpatient rehabilitation following bilateral distal femoral shortening, muscle lengthening, tendon advancement, fasciotomies, IT band releases, and peroneal nerve decompression. Preoperatively, she rapidly fatigued when independently performing activities of daily living at the wheelchair level, and had functional mobility decline due to pain limitations and deficits in endurance, balance, and coordination. On admission, the patient was dependent for bathing, toileting hygiene, lower body dressing, transfers, and manual wheelchair use; she required substantial/maximal assistance (A) for upper body dressing, partial/moderateA for bed mobility, supervision/touchingA for oral hygiene, and setup/clean-upA for eating. Walking and picking up an object were not attempted. The patient learned pain management techniques and completed progressive weight bearing, standing balance, and lower extremity joint extension therapies.  

Discussions: MYH3-associated AMC is a rare condition that causes non-progressive contractures at birth. Treatment, which often involves early surgical intervention, primarily focuses on optimizing patient quality of life (QoL) through maintaining and increasing joint range of motion, managing pain, and promoting muscle strengthening. On discharge, the patient was independent for bed mobility, sit-to-stand, chair transfers, walking, manual wheelchair use, oral hygiene, and dressing; she required setup/clean-upA for toileting hygiene, supervision/touchingA for toilet transfer and picking up an object, partial/moderateA for walking on steps, and dependent for car transfers. Walking on uneven surfaces was not attempted. 

Conclusions:

This case report, which outlines this patient’s effort toward achieving her long-term goal of higher functional independence, emphasizes the importance of post-surgical rehabilitation for AMC patients to optimize QoL. The patient made excellent gains by meeting all self-care and 11/16 mobility goals while increasing independence in all areas aside from car transfers. She was successfully discharged home and to school using a Nimbo Posterior Walker and manual wheelchair.