Distal Humerus External Rotation Osteotomy for Hand Position in Arthrogryposis

Abstract

Purpose In the amyoplasia type of arthrogryposis a reverse pronated grasp pattern is often seen. We hypothesized that repositioning the hands, through distal humerus external rotation osteotomies (DHO), would allow for palm-to-palm grasp without arm cross-over, and would improve function and parent/patient satisfaction. Methods The medical records of all patients treated surgically for arthrogryposis were reviewed at the Shriners Hospital in St. Louis, MO. From 2012–2014, nine patients (14 extremities) had undergone a DHO. All patients had pre-operative and post-operative video recordings of functional activities and we assessed functional changes after osteotomies. Pre-operative upper extremity position was graded as 1= palms facing midline, 2=palm facing posterior, and 3=palms facing away from midline. Post-operative Pediatric Outcomes Data Collection Instrument (PODCI) questionnaires were obtained and parent satisfaction was evaluated. Results Mean patient age at the time of surgery was 6.5 years-old. There were 5 patients who underwent bilateral DHOs. All patients had 3 or fewer additional procedures on an upper extremity during the study period. All patients had an improved resting posture of the upper extremity after DHO surgery, with a mean change of 51 degrees (range 15–90 degrees). Grasp pattern was altered in 13 extremities; there was a change in hand position of at least one grade and 5 had complete change from 3 to 1, palms facing away from midline to facing towards midline. There was a wide range in post-operative PODCI scores for function, but Happiness scores were high, mean 89 (range 60–100). Parents universally stated the procedure improved the child’s function “a great deal”. There were two complications, one periprosthetic humerus facture with recurrence of the internal rotation and one patient with scarring of the triceps requiring tenolysis. Conclusions The DHO is an effective procedure for correcting the internal rotation position of the upper extremity in arthrogryposis and the surgery improves hand opposition with minimal complications. Universally there was perceived improved function with high post-operative PODCI Happiness scores. Level of Evidence Therapeutic, Level V

Psychosocial wellbeing among children and adults with arthrogryposis: a scoping review

“…Communication and motivation in general increased and he was able to keep up with his siblings and was more independent in his mobility Spencer et al [ 7 ] Cross-sectional study, Level IV To document the BMD of children with Amyoplasia and predominantly lower extremity arthrogryposis and compare it with age normative values. Secondarily, to compare bone mineral density with functional ability as quantified by the PODCI and the WeeFIM and with fracture risk in patients with arthrogryposis N = 30; range 5–18 years PODCI (unclear if patient or parent report) The “happiness” normalized scores was within the norm 49 Wall et al [ 29 ] Retrospective cohort study, Level III The hypothesis is that repositioning the hands, through distal humerus external rotation osteotomies, would allow for palm-to-palm grasp without arm cross-over, and would improve function and parent/patient satisfaction N = 9; range 2–13 years; average follow-up 1.9 years; range 6 months–4 years PODCI (parent report) Post-operative mean «happiness» score: 89 (range 60–100) Wall et al [ 30 ] Cross-sectional study (part of a larger longitudinal cohort study) Level IV To investigate upper extremity functional and psychosocial measures in patients with arthrogryposis, comparing Amyoplasia and distal only involvement, utilizing the PROMIS and PODCI questionnaires Amyoplasia N = 15; range, 6–10 years. Distal arthrogryposis N = 14; range 8–12 years PODCI; PROMIS (UE function, pain interference, depression, anxiety, and peer relations) Median “happiness” scores: All subjects (N = 29): 85 (70–95) Distal arthrogryposis (N = 14): 88 (80–99) Amyoplasia (N = 15): 85 (70–95) p = 0.40 Median PROMIS scores were in the normal range for both groups: Distal arthrogryposis: pain (46); depression (53); anxiety (51); peer relations (46) Amyoplasia: pain (50), depression (47), anxiety (49), and peer relations (56) “Peer relations” score was statistically lower for the distal arthrogryposis group Studies on adults with AMC Altiok et al [ 36 ] Cross-sectional study, Level IV To describe demographics and QOL, life satisfaction and functional mobility of young adults with AMC after transition from pediatric care N = 23 range 18–36 years SWLS, PROMIS-57 v2.0 profile, PROMIS Global health profile 56% were ...…”

Muscle and joint function in children living with arthrogryposis multiplex congenita: A scoping review

“…After a combination of triceps lengthening and capsulotomy, Van Heest et al () reported an improvement in the arc of passive motion of all 29 elbows from an average of 32° (range, 0–75°) preoperatively to an average of 66° (range, 10–125°) postoperatively. Wall et al () describes distal humerus external rotation osteotomy to improve grasp pattern among nine children with Amyoplasia with positive results.…”

Treatment and outcomes of arthrogryposis in the upper extremity

“…A few of the common patient reported outcome measures used to understand the changes in health for pediatric patients include the Pediatric and Adolescent Outcome Data Collection Instrument (PODCI) (Daltroy, Liang, Fossel, & Goldberg, ), the Pediatric Qualify of Life Scale (PedsQL) (Varni, Seid, & Rode, ), and more recently the Patient Reported Outcome Measure Information System (PROMIS) developed by the National Institute of Health (Cella et al, ; Riley et al, ). These validated measures have been utilized in the treatment of upper extremity ailments to assess the overall function, physical, mental, and social ailments of the patient (Amor, Spaeth, Chafey, & Gogola, ; Bell et al, ; James, Abarca, & Manske, ; Lerman, Sullivan, Barnes, & Haynes, ; Wall et al, ).…”

“…At this time, there are only two studies that specifically address the internal rotation posturing of the shoulder (Ramirez, Richards, Kozin, & Zlotolow, ; Wall et al, ). These two studies both advocate for humeral rotational osteotomies to address the internal rotation contracture or posture of the shoulder.…”

“…Obviously, the goal of any surgical procedure is to augment upper extremity function and performance of activities of daily living (ADL's) (Abdel‐Ghani, Mahmoud, Shaheen, & Abdel‐Wahed, ; Nouraei, Sawatzky, MacGillivray, & Hall, ; Wall, Calhoun, Roberts, & Goldfarb, ). Treatment must be individualized, and various approaches have been described to address the deformities.…”