Modified osteotomy for treatment of forearm deformities (Masada IIb) in hereditary multiple osteochondromas: a retrospective review

Purpose Approximately 30% of patients with hereditary multiple osteochondromas (HMO) have forearm deformity and dysfunction. The aim of this retrospective study was to review our experience with the surgical treatment of children with HMO and Masada IIb forearm deformities. Methods Data of eight children treated for HMO Masada IIb forearm deformity at our hospital between 2015 and 2019 were collected from the hospital records and retrospectively reviewed. All patients underwent ulnar lengthening by distraction osteogenesis using either the Orthofix or Ilizarov external fixator. Range of movements at the elbow and wrist joints, and forearm supination/pronation, before and after the operation were recorded. Radiographs were evaluated by the Fogel method, and wrist joint function by the Krimmer method. Results Follow-up radiographs showed significant improvement in relative ulnar shortening after treatment (pre-operative 9.23 ± 5.21 mm; post-operative 0.33 ± 4.13 mm). Changes in radial articular angle (pre-operative 33.55° ± 3.88° to 32.78° ± 6.57°) and carpal slip (pre-operative 45.00% ± 19.09%; post-operative 43.13% ± 16.68%) were not significant. Elbow flexion and extension, wrist flexion and extension, ulnar and radial deviation at wrist, and forearm rotation were significantly improved after surgery. Wrist function was graded as excellent in seven patients and as good in one patient. One patient treated with the Ilizarov external fixator had poor radial head reduction. Conclusion Ulnar lengthening with distraction osteogenesis is an effective treatment for HMO Masada IIb deformities. The optimum site for ulnar osteotomy appears to be at the proximal one-third to one-fourth of the ulna.

Modified Osteotomy for Treatment of Forearm Deformities (Masada IIb) in Hereditary Multiple Osteochondromas: A Retrospective Review

Purpose: Approximately 30% of patients with hereditary multiple osteochondromas (HMO) have forearm deformity and dysfunction. The aim of this retrospective study was to review our experience with the surgical treatment of children with HMO and Masada IIb forearm deformities.Methods: Data of eight children treated for HMO Masada IIb forearm deformity at our hospital between 2015 and 2019 were collected from the hospital records and retrospectively reviewed. All patients underwent ulnar lengthening by distraction osteogenesis using either the Orthofix or Ilizarov external fixator. Range of movements at the elbow and wrist joints, and forearm supination/pronation, before and after the operation were recorded. Radiographs were evaluated by the Fogel method, and wrist joint function by the Krimmer method.Results: Follow-up radiographs showed significant improvement in relative ulnar shortening after treatment (pre-operative 9.23 ± 5.21 mm; post-operative 0.33 ± 4.13 mm). Changes in radial articular angle (pre-operative 33.55° ± 3.88° to 32.78° ± 6.57°) and carpal slip (pre-operative 45.00% ± 19.09%; post-operative 43.13% ± 16.68%) were not significant. Elbow flexion and extension, wrist flexion and extension, ulnar and radial deviation at wrist, and forearm rotation were significantly improved after surgery. Wrist function was graded as excellent in seven patients and as good in one patient. One patient treated with the Ilizarov external fixator had poor radial head reduction.Conclusion: Ulnar lengthening with distraction osteogenesis is an effective treatment for HMO Masada IIb deformities. The optimum site for ulnar osteotomy appears to be at the proximal one-third to one-fourth of the ulna.

Ulnar osteotomy is the only required procedure for chronic monteggia fracture in children.

Objective: To determine containment of radial head after ulnar osteotomy in chronic Monteggia fractures. Study Design: Retrospective study. Setting: Children Hospital and The Institute of Child Health, Lahore. Period: 2019 to January 2020. Material & Methods: Ten patients presented in outdoor patient department with a diagnosis of chronic Monteggia fracture. Four patients were labelled as missed Monteggia fractures, four with late presented Monteggia fractures and two with inadequately treated Monteggia fractures. Bado and Letts classifications were applied for patients. Mean age was 6 years and 8 months and ranged from 4 to 10 years. Mean time interval between injury and admission was 6.1 months. Open reduction of radial head and ulnar osteotomy was done through Boyed approach. Transcapitellar wire was inserted temporarily and then removed so it is not required permanently. The ulnar osteotomy was angulated opposite to the direction of radial head dislocation and fixed with plate and screws. Results: 10 patients were included in this study. The age range was between 4 and 10 years. The study period was six months and follow up was one year. Mean ulnar angulation at osteotomy site was 21.3° (16-25°). Mean ulnar lengthening at osteotomy site was 0.85 cm (0.5-1.8 cm).Improvement in flexion-extension was 20.3%, pronation was 5.1% and supination was 13.7%. Complications included were nounion in 1 case and cubitus valgus in 1 case. Conclusion: Containment of radial head is obtained by open reduction of radial head and with ulnar osteotomy in chronic Monteggia fractures. Annular ligament reconstruction and transcapitellar wire insertion are not required if proper angulation and fixation of ulnar osteotomy is performed. No age limit for this procedure but surgery should be performed before radial head deformation.

A new technique for neonatal Jeune syndrome: External thoracic expansion

Jeune syndrome is a rare form of skeletal dysplasia characterized by a narrow, bell-shaped chest (thoracic cage), and typical phalangeal and pelvic bone deformities. Chest expansion is impaired by the short, horizontally positioned ribs, resulting in alveolar hypoventilation and eventually neonatal-infantile death in most cases. External distraction with sternoplasty is a new technique for the treatment of Jeune syndrome, which was firstly used by our team on a newborn by placing a sliding finger fixator which was designed for ulnar lengthening. We believe that this approach can be life-saving in neonates with improved and widespread usage.

Gradual ulnar lengthening in Masada type I/IIb deformity in patients with hereditary multiple osteochondromas: a retrospective study with a mean follow-up of 4.2 years

Background Gradual ulnar lengthening is the most commonly used procedure in the treatment of Masada type I/II deformity in patients with hereditary multiple osteochondromas. However, the treatment remains controversial for the recurrence of deformity in growing children. This study aims to evaluate the clinical and radiological outcomes of ulnar gradual lengthening in our clinic. Methods We retrospectively reviewed patients who underwent ulnar lengthening by distraction osteogenesis from June 2008 to October 2017. The carrying angle (CA) and range of motion (ROM) of the forearm and elbow were clinically assessed, and the radial articular angle (RAA) and ulnar shortening (US) were radiologically assessed before lengthening, 2 months after external frame removal, and at the last follow-up. Results The current study included 15 patients (17 forearms) with a mean age of 9.4 ± 2.3 years at the index surgery. The mean follow-up period was 4.2 ± 2.4 years. There were 9 patients (10 forearms) with Masada type I deformity and 6 patients (7 forearms) with Masada type IIb deformity. The mean amount of ulnar lengthening was 4.2 ± 1.2 cm. The mean RAA improved from 37 ± 8 to 30 ± 7° initially (p = 0.005) and relapsed to 34 ± 8° at the last follow-up (p = 0.255). There was a minimal deterioration of US yet significant improvement at the last follow-up compared to pre-op (p < 0.001). At the last follow-up, the mean forearm pronation and elbow flexion increased significantly (p < 0.001 and p = 0.013, respectively), and the mean carrying angle also improved significantly (p < 0.001). No patient with type IIb deformity achieved a concentric radial head reduction. Conclusions Gradual ulnar lengthening significantly reduces cosmetic deformity and improves function in patients with Masada type I/IIb deformity. Our results supported early ulnar lengthening for patients with a tendency of dislocation of the radial head.

Older age and multi-joint external fixator are two risk factors of complications in ulnar lengthening in children with hereditary multiple exostosis

Objectives Hereditary multiple exostosis (HME) often involves forearm deformities. The aim of this study was to present the clinical results of 37 children who underwent ulnar lengthening with two different types of unilateral external fixators and to investigate the risk factors of complications. Methods We evaluated 37 children with forearm deformities caused by HME treated in our hospital from January 2008 to July 2019. The surgical procedures included resection of exostosis, osteotomy of the ulna, and gradual lengthening of the ulna with a unilateral external fixator. According to the type of fixator they received, the children were divided into two groups: group A received monorail fixators and group B received multi-joint fixators. Radiographic and functional parameters were assessed. Complications were recorded. Results All patients were followed-up for an average of 4.6 years (3.0 to 6.5). In both group A and group B, the ulna shortening (US), radial articular angle (RAA), carpal slip (CS), elbow flexion, forearm pronation, supination, and Mayo Elbow Performance Score (MEPS) values improved significantly from preoperatively to postoperatively (p < 0.05). However, the ulnar deviation was observed in 4 cases in group B and no cases in group A. According to logistic regression, the difference was only related to age (p < 0.05) and the type of external fixator (p < 0.05). Conclusions Ulnar lengthening with unilateral external fixation is a safe and effective procedure for the treatment of HME. Regarding complications, deviation of the ulna axis was more likely to occur in older children with multi-joint external fixators.

A Commentary on “Clinical and Functional Outcomes of Ulnar Lengthening in the Treatment of Masada Type I Forearm Deformities in Hereditary Multiple Osteochondromas”

The article's abstract is not available.

Older age and multi-joint external fixator are two risk factors of complications in ulnar lengthening in children with hereditary multiple exostosis

Objectives: Hereditary multiple exostosis (HME) often involves forearm deformities. The aim of this study was to present the clinical results of 37 children who underwent ulnar lengthening with two different types of unilateral external fixators, and to investigate the risk factors of complications. Methods: We evaluated 37 children with forearm deformities caused by HME treated in our hospital from January 2008 to July 2019. The surgical procedures included resection of exostosis, osteotomy of the ulna, and gradual lengthening of the ulna with a single arm external fixator. According to the type of external fixator they received, the children were divided into two groups: group A received monorail fixators, and group B received multi-joint fixators. Radiographic and functional parameters were assessed. Complications were recorded. Results: All patients were followed up for an average of 4.6 years (3.0 to 6.5). In both group A and group B, the ulna shortening (US), radial articular angle (RAA), carpal slip (CS), elbow flexion, forearm pronation, supination, and Mayo Elbow Performance Score (MEPS) values improved significantly from preoperatively to postoperatively ( p < 0.05). However, ulnar deviation was observed in 4 cases in group B and no cases in group A. According to logistic regression, the difference was only related to age ( p <0.05) and the type of external fixator ( p <0.05). Conclusions: Ulnar lengthening with unilateral external fixation is a safe and effective procedure for the treatment of HME. Regarding complications, deviation of the ulna axis was more likely to occur in older children with multi-joint external fixators.

Management of Masada type I/IIb forearm deformity by gradual ulnar lengthening in patients with hereditary multiple osteochondromas

Background Gradual ulnar lengthening is the most commonly used procedure in treatment of Masada type I/II deformity in patients with hereditary multiple osteochondromas. However, the treatment remains controversial for the recurrence of deformity in growing children and only slightly handicapped of daily life activities in untreated adult patients. The aim of this study was to evaluate mid-term clinical and radiological outcomes of ulnar gradual lengthening in our clinic. Methods We retrospectively reviewed patients who underwent ulnar lengthening by distraction osteogenesis from June 2008 to October 2017. Patients with less than two years of follow-up were excluded. The surgical procedures consisted of ulnar lengthening by external fixator and/or excision of the osteochondroma at the distal ulna. The carrying angle (CA) and range of motion (ROM) of the forearm and elbow were clinically assessed, and the radial articular angle (RAA) and ulnar shortening (US) were radiologically assessed before lengthening, two months after external frame removal, and at the last follow-up. Results The current study included 15 patients (17 forearms) with a mean age of 9.4 ± 2.3 years at index surgery. The mean follow-up period was 4.2 ± 2.4 years. There were 9 patients (10 forearms) with Masada type I deformity and 6 patients (7 forearms) with Masada type IIb deformity. The mean amount of ulnar lengthening was 4.2 ± 1.2 cm. The mean RAA improved from 37.3 ± 7.9° to 29.8 ± 6.5° initially (P = 0.003) and relapsed to 34.4 ± 7.6° at the last follow-up (p = 0.234). There was minimal deterioration of US yet significant improvement at the last follow-up compared to pre-op (p < 0.001). The elbow flexion and forearm pronation were improved significantly at last follow-up (p < 0.001 and p = 0.024, respectively). The mean carrying angle improved significantly from − 6.3 ± 7.4 ° preoperatively to 7.4 ± 11.1° at last follow-up (p < 0.001). Conclusions Gradual ulnar lengthening significantly reduces cosmetic deformity and improves function; it is recommended for patients with Masada type I/IIb deformity. Therefore, we advocated aggressive individual treatment protocol for patients with Masada type I/IIb deformities.