Application of C-Shaped Osteotomy and Distraction Osteogenesis for Correction of Radial Angulation Deformities of the Hand in Children With Apert Syndrome

Abstract

Apert syndrome is characterized by short, radially deviated thumbs, leading to difficulties in daily life such as holding a fork or a spoon and buttoning up. The main goal of surgery is to achieve thumb to index finger pinch to overcome these difficulties. Seven patients (14 extremities) followed up with Apert syndrome underwent distraction after a C-shaped osteotomy to simultaneously correct the brachydactyly and the angulation deformity of the bilateral thumbs. The patients ranged in age from 4 to 7 years at the distraction operation, with a mean (SD) of 4.7 (1.7) years, and the mean (SD) length of follow-up was 100.6 (14.95) months. The mean (SD) length of the phalanges at the beginning of distraction was 19.1 (3.26) mm, and the mean (SD) length of the distracted phalanx at long-term follow-up visit was 26.2 (5.63) mm. The mean (SD) correction of radial angulation was calculated as 42.6 (9.95) degrees, and the difference was considered as being statistically significant (P < 0.001). Minor complications such as pin loosening were observed in 6 extremities of 4 patients, and 2 patients were treated for pin tract infection. Index-to-thumb pinching was acquired by all the patients after distraction and correction of angulation. The hand features of Apert syndrome are as noteworthy as the craniofacial features and thus may lead to functional impairment and limitations in daily life. Distraction with a C-shaped osteotomy seems to be a promising method to correct both the shortness and the radial angulation of the thumb to achieve functional results.

Distraction Lengthening of the Apert Thumb

“…This aligns with limited pediatric studies demonstrating broad functional improvement after distraction. 11,13 Study limitations must be acknowledged. This is a retrospective study performed by a single group over an extended time.…”

“…Studies show that minor complications (e.g., pin site infections) are common during distraction, occurring in patients who undergo callotasis or the two-stage appro ach. 11,19,32,[34][35][36] Although major complications are more common in the two-stage approach compared to callotasis, they are typically rare. 19,32,34,37,38 In our cohort, major complications, such as malunion and delayed union, occurred in one patient each.…”

Apert Syndrome: New treatment and a perspective for the future

“…The main goal of surgery for hand anomalies of Apert syndrome patients is to reconstruct the thumb to index finger pulp-topulp pinch to overcome common difficulties in their daily life such as holding a fork or a spoon and buttoning up. [57,58]. Various classifications have been used to assist surgeons in planning surgery.…”

“…Because radial deviation of the thumb is associated with the loss of index-to-thumb pulp-to-pulp pinch, these patients may experience limitations in their daily life activities such as problems in buttoning up and holding a spoon. [63,58].…”

“…This is probably related to the distraction and adaptation of the soft tissue as well. [58] Possible Treatment In The Future?…”