Long-term results of surgery for forearm deformities in patients with multiple cartilaginous exostoses

BACKGROUND: Surgical treatment of forearm deformities in patients with multiple cartilaginous exostoses remains controversial. The purpose of the present study was to determine the reasonable indications for operative treatment and to evaluate long-term results of forearm surgery in these patients. METHODS: We retrospectively reviewed twenty-three patients (thirty-one forearms) after a mean duration of follow-up of nearly thirteen years. The mean age at the time of the initial procedure was eleven years. The patients underwent a variety of surgical procedures, including excision of exostoses; corrective procedures (lengthening of the radius or ulna and/or corrective osteotomy of the radius and/or ulna) and open reduction or excision of a dislocated radial head. Clinical evaluation involved the assessment of pain, activities of daily living, the cosmetic outcome, and the ranges of motion of the wrist, forearm, and elbow. The radiographic parameters that were assessed were ulnar variance, the radial articular angle, and carpal slip. RESULTS: Four patients had mild pain, and five patients had mild restriction of daily activities at the time of follow-up. Eight patients stated that the appearance of the forearm was unsatisfactory. Radiographic parameters (ulnar variance, radial articular angle, carpal slip) were initially improved; however, at the time of the final follow-up visit, the deformities had again progressed and showed no significant improvement. The only procedure that was associated with complications was ulnar lengthening. Complications included nonunion (three forearms), fracture of callus at the site of lengthening (two forearms), and temporary radial nerve paresis following an ulnar distraction osteotomy (one forearm). Excision of exostoses significantly improved the range of pronation (p = 0.036). CONCLUSIONS: In our patients with multiple cartilaginous exostoses, corrective osteotomy and/or lengthening of forearm bones was not beneficial. The most beneficial procedure was excision of exostoses. Reasonable indications for forearm surgery in these patients are (1) to improve forearm rotation and (2) to improve the appearance.     

Correction and lengthening for deformities of the forearm in multiple cartilaginous exostoses

Background Multiple cartilaginous exostoses cause various deformities of the epiphysis. In exostoses of the ulna, the ulna is shortened and the radius acquires varus deformity, which may lead to dislocation of the radial head. In this study, we present the results of exostoses resection, with correction and lengthening with external fixators for functional and cosmetic improvement, and prevention of radial head dislocation. Methods We retrospectively reviewed seven forearms of seven patients who had deformities of the forearm associated with multiple cartilaginous exostoses. One patient had dislocation of the radial head. Operative technique was excision of osteochondromas from the distal ulna, correction of the radius, and ulnar lengthening with external fixation up to 5 mm plus variance. We evaluated radiographs and the range of pronation and supination. Furthermore, we conducted a follow-up of ulnar length after the operation. Results Dislocation of the radial head of one patient was naturally reduced without any operative intervention. At the most recent follow-up, six of the seven patients showed full improvement in pronation–supination. Ulnar shortening recurred with skeletal growth of four skeletally immature patients; however, it did not recur in one skeletally mature patient. Overlength of 5 mm was negated by the recurrence of ulnar shortening about 1.5 years after the operation. Conclusions We treated seven forearms of seven patients by excision of osteochondromas, correction of radii, and gradual lengthening of ulnas with external fixators. The results of the procedure were satisfactory, especially for function of the elbow and wrist. However, we must consider the possible recurrence of ulnar shortening within about 1.5 years during skeletal growth periods in immature patients.     

Lengthening the ulna in patients with hereditary multiple exostoses

Deformity of the forearm is common in patients with hereditary multiple exostoses, producing cosmetic and functional impairment in which shortening of the ulna is a significant factor. The results of ulnar lengthening in 10 forearms of eight patients are reported. Lengthening was performed by osteotomy of the shaft followed immediately by a bone graft and internal fixation, or by gradual distraction with an external fixator. In all patients the appearance was improved and the range of radial deviation at the wrist was increased. In most patients forearm movement and radial head stability were improved. Partial recurrence of the deformity was seen during the follow-up of skeletally immature patients, but in general ulnar lengthening was found to be a useful operation.     

Management of deformities of the forearm in multiple hereditary osteochondromas

A patient with multiple hereditary osteochondromas may have any of several severe deformities of the forearm, the most common of which are ulnar deviation of the wrist associated with relative shortening of the ulna, bowing of either or both of the bones of the forearm, shortening of the forearm, and late dislocation of the radial head. The natural history of these deformities is progression, with variable weakness, functional impairment, and cosmetic deformity of the extremity. We describe the results, after a follow-up of at least two years, in eighteen patients who underwent major surgical procedures: excision of the osteochondromas (ten patients), ulnar lengthening with excision of the osteochondromas (three patients), and ulnar lengthening with radial hemiepiphyseal stapling (seven forearms of five patients). Early excision of the osteochondromas alone did not slow the progression of the deformity. Ulnar lengthening did, on occasion, correct the ulnar drift at the wrist, but the relative shortening of the ulna recurred. Distal radial hemiepiphyseal stapling should accompany ulnar lengthening if radiocarpal angulation or subluxation of the lunate occurs with ulnar shortening. Deformities of the forearm should be treated early and aggressively to prevent disability.     

The Taniguchi classification in cases of multiple cartilaginous exostoses]

27 patients treated surgically at Child Orthopaedic Clinic of Pomeranian Medical Academy between 1974-1996 for multiple cartilaginous exostosis (Aclasia Diaphysealis Keith) were classified into three groups according to the Taniguchi classification. This classification is based on whether multiple cartilaginous exostoses are present on distal forearm. Group I--no involvement of the distal forearm (n = 2), in group II involvement of the distal forearm without shortening of either bone (n = 7) was stated. Group III consists of members with involvement of the distal forearm with shortening the radius or the ulna (n = 18). Groups were compared with regard to: number of lesions, distribution of exostoses in different body areas, age of onset of the Keith disease, height of children, presence of valgus deformity of the ankle, dislocation of the radial head and presence of exostoses around hip area. This classification should be useful in estimating severity of Keith disease, identifying cases at high risk for complications like dislocation of the radial and malignant transformation.     

Correction of forearm deformities in children with multiple cartilaginous osteochondromas

AIM: Deformity of the forearm with shortening and bowing is common in children with multiple cartilaginous osteochondromas. The objective of this study was to evaluate the benefit of ulnar lengthening using an external fixateur in these patients. METHOD: 9 patients (10 cases) underwent surgery of the forearm between 1995 and 2001 and were evaluated using a standard protocol. The mean follow-up was 33.6 months, the mean age at operation 8.9 years. All patients were treated with ulnar lengthening, in 6 cases combined with an excision of the osteochondromas. RESULTS: Four out of ten patients did show an improvement in postoperative forearm rotation, two deteriorated and 4 presented unchanged. Wrist motion improved in 7 patients and remained unchanged in 3. The postoperative radial articular angle showed an improvement in 6, the carpal slip in 9 of the patients. The preoperative radial head dislocation in one patient remained unchanged postoperatively. CONCLUSION: The authors advocate this therapeutic concept for the correction of forearm deformity in multiple hereditary osteochondromas to prevent a progression of the deformity and to establish carpal stability. A significant improvement of forearm and wrist function could not be reached.     

Forearm lengthening using Ilizarov external fixator

Background

We present our experience with forearm lengthening using Ilizarov external fixator in cases of length discrepancies between radius and ulna and forearm–wrist deformity that occurred duo to different causes.

Methods

Twelve patients were treated by Ilizarov external fixator between 2008 and 2010 with a mean age of 10 years. There were seven males and five females. The etiology was Madelung’s deformity in seven patients, multiple cartilaginous exostosis in three patients, post-traumatic growth arrest of distal radius epiphysis in one patient, and chronic osteomyelitis of radius in one patient. The length discrepancy ranged from 1 to 4 cm (mean 2.2 cm). Lengthening of radius was done in nine patients and lengthening of ulna in three patients. The mean of follow-up period was 2 years.

Results

At follow-up, all patients were satisfied with the functional and cosmetic results. There was an improvement in pain and range of motion .The mean length gained was 2.2 cm and the mean healing index was 43.7 days/cm.

Conclusions

Lengthening of short forearm is functionally, cosmetically, and psychologically beneficial. The Ilizarov method is a reliable, successful, and safe method, and it is the gold standard to treat forearm length discrepancy and deformity problems preserving a satisfactory function of upper limb during treatment.     

Operations for forearm deformity caused by multiple osteochondromas

We reviewed 36 cases of forearm deformity caused by multiple osteochondromas in 30 patients and classified them into three types: Type I showed a combination of ulnar shortening and bowing of the radius secondary to osteochondromas of the distal ulna (22 forearms). Type II showed dislocation of the radial head, either with osteochondromas of the proximal radius (Type IIa, two forearms) or secondary to more distal involvement (Type IIb, five forearms). Type III had relative radial shortening due to osteochrondromas at the distal radius (seven forearms). Operations were performed on 16 forearms in 13 patients, with 92% of satisfactory results. For Type I deformity, excision of osteochondromas, immediate ulnar lengthening and corrective osteotomy of the radius are recommended. For Type IIa, excision of the radial head is necessary, and for Type IIb, we advise gradual lengthening of the ulna using an external fixator. Excision of osteochondromas alone gave good results in Type III deformity. Our classification gives a reliable indication of the prognosis and is a guide to the choice of surgical treatment.     

Tumor location affects the results of simple excision for multiple osteochondromas in the forearm

BACKGROUND: The effectiveness of excision of osteochondromas in controlling the progression of forearm and wrist deformity remains an issue of controversy. The purpose of this study was to analyze the effectiveness of tumor excision in the correction of forearm and wrist deformity due to multiple osteochondromas in children, with an interpretation of the results based on different patterns of deformity. METHODS: Fourteen forearms in thirteen children with a follow-up of more than twenty-four months (average, fifty-three months) were included in the study. The forearms were divided into two groups on the basis of the location of the tumor and the pattern of deformity. In Group 1 (six forearms), the osteochondroma was only in the distal aspect of the ulna and caused compression of the radius. In Group 2 (eight forearms), tumors were in both the distal aspect of the ulna and the ulnar side of the distal part of the radius and were in contact with each other. Radial length, ulnar shortening, radial bowing, the radial articular angle, and carpal slip were measured as radiographic parameters. Ulnar shortening and radial bowing were expressed as a percentage of the radial length to make it possible to compare data between the individuals. Each parameter was evaluated before surgery and at the time of final follow-up. RESULTS: In Group 1, the percentage of ulnar shortening and the percentage of radial bowing had improved at the time of final follow-up; however, in Group 2, both the radial articular angle and the percentage of radial bowing had deteriorated significantly after the tumor excision (p = 0.049 and p = 0.017, respectively), even though the percentage of ulnar shortening showed no change. CONCLUSIONS: The effectiveness of simple excision of osteochondromas of the distal aspect of the forearm is influenced by the tumor location and is related to the pattern of the deformity. Simple tumor excision can correct the forearm deformity in patients with an isolated tumor of the distal part of the ulna. Conversely, in patients with tumors involving the distal part of the ulna and the ulnar side of the distal end of the radius, tumor excision alone is a less promising procedure for the correction of the deformity. LEVEL OF EVIDENCE: Prognostic Level IV.     

外固定架尺骨延长治疗遗传性多发性骨软骨瘤所致前臂畸形

目的探讨采用外固定架尺骨延长治疗遗传性多发性骨软骨瘤(HMO)所致前臂畸形的疗效。方法回顾分析2014年4月至2018年4月北京积水潭医院创伤骨科采用外固定架尺骨延长治疗HMO所致前臂畸形患者5例。其中男4例,女1例;平均年龄14.5岁(12~16岁);右侧2例,左侧3例;MasadaⅠ型2例,MasadaⅡB型3例。5例患者均接受尺骨延长手术治疗。对3例MasadaⅡB型患者采用环形外固定架,另2例采用单边外固定架。1例合并桡骨骨折患者同时行桡骨骨折切开复位内固定术。截骨术后8~10 d开始行尺骨牵开延长。结果5例患者均获得随访,平均随访时间16个月(12~30个月)。3例MasadaⅡB型患者桡骨头均自行复位。5例患者尺骨平均延长37.5 mm(30~45 mm),平均应用外固定架时间162 d(122~274 d),平均外固定架指数48.9 d/cm。术前和术后平均梅奥肘关节评分分别为36.4分和92.7分,平均肘关节屈曲活动范围分别为118.0°(110°~130°)和130.0°(120°~150°),平均伸肘活动范围分别为12.7°(10°~20°)和3.5°(0°~10°),平均前臂旋前活动范围分别为18.6°(5°~30°)和44.7°(30°~65°),平均前臂旋后活动范围分别为71.2°(50°~85°)和86.5°(75°~90°)。1例桡骨干骨折患者术后3个月骨折愈合。2例出现针道感染;1例尺骨过早愈合,行第2次截骨手术后延长顺利。未见神经血管并发症。5例患者对治疗结果均满意。结论采用外固定架逐渐延长尺骨治疗HMO患者尺骨短缩畸形和桡骨头脱位安全有效。     

Management of forearm deformities with ulnar shortening more than 15 mm caused by hereditary multiple osteochondromas

Background

Treatment for forearm deformities caused by hereditary multiple osteochondromas is still controversial. The purpose of the study was to describe the outcome of treatment with the combined use of ulnar lengthening and excision of osteochondromas.

Methods

Fourteen patients with hereditary multiple osteochondromas had forearm deformities which belongs to type I Masada deformity. The mean relative ulnar shortening was 19.9 mm (range, 16–23). All patients ranging from 4 to 15 years old had operative treatment. Clinical evaluation involving the assessment of pain, activities of daily living, and range of motion of the wrist and forearm was performed. The radiographic evaluation was relative ulnar shortening (RUS), radial articular angle (RAA), carpal slip (CS), and radial bowing. Besides, we also evaluated the amount of ulnar lengthening, external fixation time, and external fixation index.

Results

The mean EFT was 101 days (range, 84–133), and the mean external fixation index was 44.4 days/cm (range, 33.6–51.2). During the mean 43.1 months follow-up, four patients had mild pain and two patients had mild restriction of daily activities. The rotation of forearm and motion of wrist had increased except in Case 3. The RAA, CS, and radial bowing had been improved except in Case 6 and 7. In all patients, relative ulnar shortening had been corrected. According to the functional evaluation criteria recommended by Krimmer, there were ten excellent and four good.

Conclusions

The combined use of ulna lengthening and excision of osteochondromas is a proper and effective treatment method to the type I Masada deformity with the relative ulnar shortening more than 15 mm.     

Ulnar focal cortical indentation: a previously unrecognised form of ulnar dysplasia

Deformity of the forearm due to growth disturbance of the ulna occurs in a number of conditions such as ulnar deficiency, multiple exostoses, and neurofibromatosis. We report a previously unrecognised form, caused by focal cortical indentation.We have treated five children with this condition, three girls and two boys; the mean age at presentation was 5 years (2 to 8). The deformity was first recognised about the age of two years, and progressed gradually. The radiological findings were the same in all cases. The focal cortical indentation was seen at the distal end of the ulna with anteromedial bowing and dysplasia. The radial head was dislocated posterolaterally. In one patient the histological findings at the site of indentation were of a fold of tissue resembling periosteum, which interfered with enchondral ossification. Treatment by ulnar lengthening using an external fixator and osteotomy which corrected both the ulnar deformity and reduced the dislocated radial head in two cases gave the best results.     

尺骨干骺端续连症不同手术方法的疗效观察

目的:探讨尺骨骨软骨瘤切除、尺骨微创截骨、外固定尺骨延长术治疗尺骨干骺端续连症前臂畸形治疗效果和安全性。方法:自2005年8月至2013年12月,20例尺骨干骺端续连症患者,男15例,女5例;年龄7~13(10.00±2.34)岁;病程6~11(8.10±1.52)个月;临床表现为患侧前臂短缩并向尺侧弯曲畸形。采用尺骨骨软骨瘤切除、尺骨微创截骨、外固定尺骨延长术治疗,术后评估包括评估疼痛、日常生活活动、外观矫形效果及腕关节、肘关节和前臂的运动范围,放射学评估包括尺骨长度、桡骨关节面倾斜角和腕部骨骺生长情况。结果:所有患者术后伤口愈合,未出现感染,与并发症相关的惟一术式是尺骨延长,包括1例骨不连、2例尺骨延长骨痂骨折、1例暂时性的桡神经麻痹。所有患者获得随访4~7.5(6.03±1.33)年。所有病例腕关节桡偏尺偏活动度数、前臂旋前旋后度数变化差异有统计学意义(P0.05),放射学评估参数(尺骨方差,桡骨关节角,腕骨滑动)得到了改善且差异具有统计学意义(P0.05),末次随访时改良Green和O'Brien腕关节功能评分与术前比较差异有统计学意义(P0.05),腕关节的临床疗效与术前比较差异有统计学意义(P0.05),末次随访Mayo肘关节功能评分与术前比较差异有统计学意义(P0.05),肘关节的临床疗效与术前比较差异有统计学意义(P0.05)。结论:尺骨骨延长对阻止远期畸形的进展无益,单纯的尺骨远端骨软骨瘤切除术有利于阻止畸形的发展,腕关节和前臂旋转活动受限及对外观的改善有强烈的要求的患者可积极进行手术治疗。     

The association between ulnar length and forearm movement in patients with multiple osteochondromas

PURPOSE: To determine the relationship between the length of the ulna as a proportion of height (proportional ulnar length [PUL]), forearm and wrist ranges of motion, and degree of observable deformity in people with hereditary multiple exostoses. METHODS: One hundred forty-two people with hereditary multiple exostoses were examined; 35 were under the age of 15 years and therefore were presumed to be skeletally immature. Elbow, forearm, and wrist motion were measured, and the radius and ulna were palpated for osteochondromas. Ulnar length was estimated as a proportion of height (PUL) in skeletally immature subjects. The relationships between total active motion, number of palpable osteochondromas, and proportional length were examined for one randomly selected limb from each subject. RESULTS: A negative correlation was found between the number of palpable osteochondromas and range of forearm rotation. The degree of forearm motion in those under the age of 15 years was directly related to PUL and indirectly related to the number of palpable osteochondromas. Children whose PUL is within the normal range have a normal range of motion. CONCLUSIONS: In a child with hereditary multiple exostoses affecting the forearm, the PUL is associated with the range of movement and deformity, and it can be a useful adjunct in deciding the appropriate management.     

Reconstruction of the DRUJ in a young adult after resection of a large exostosis of the distal radius

The prevalence of known solitary exostosis is around 1–2 % in the general population. Treatment of an exostosis may consist of resection with or without further treatment for deformity. The distal radioulnar joint (DRUJ) acts as the link between radius and ulna at the wrist and is important in the transmission of load. Its anatomic integrity should be respected in surgical procedures or ulnar-sided wrist pain because of instability, limitation of forearm rotation and potential development of grip weakness may develop. We present a case of reconstruction of the DRUJ with distraction lengthening of the ulna after resection of a large exostosis of the distal radius that had resulted in a malformed and dysplastic ulna. This treatment in a young patient resulted in a stable, functional and congruent distal radioulnar joint.     

The treatment of hereditary multiple exostosis of the upper extremity

Thirty of 50 patients with hereditary multiple exostosis developed significant deformities of the arm in one extremity. The degree of deformity is dependent on the location of the osteochondroma. If the osteochondroma is on the radius, deformity will usually be only minimal. If the osteochondroma is at the distal end of the ulna, the epiphysis usually stops growing. We believe that the ulnar collateral ligament then acts as a tether, very similar to that seen in the ulnar clubhand. The radius then has to either bow or dislocate at the elbow, and the wrist displaces ulnarly. We performed operations on 10 patients that consisted of cutting the ulnar collateral ligament, lengthening the ulna, osteotomizing the radius, and removing any osteochondromas. In the young, growing child, staples are placed across the lateral side of the distal radial epiphysis. The cosmetic results of the surgery were very gratifying. Nine patients also had osteochondromas removed from the hands and the forearms.     

Madelung deformity: isolated ulnar wedge osteotomy

OBJECTIVES: Madelung deformity is defined as a partial closure of the medial half of the distal radial growth plate, with anatomical consequences (wrist deformity), and functional impairment (decrease range of motion, loss of grip strength, and wrist pain). We report a new surgical procedure including a shortening of the ulna combined with slight anterior flexion osteotomy aiming to correct the radio-ulnar dislocation and to improve the range of forearm rotation, without correcting the global deformity. METHODS: This was a retrospective study of four symptomatic wrists in three patients. The follow-up was 24 months. Patients were assessed clinically and radiologically pre and post operatively. The ulnar shaft was shortened and anteriorly flexed through a dorsal and ulnar approach and fixed with a fitted internal DCP plate. The aim of this osteotomy was to reduce the dislocated distal radio-ulnar joint. RESULTS: At 24 months follow-up, the function was considerably improved with a correction of the ulno-carpial conflict. The range of painfree forearm rotation improved. The post-operative views showed a correction of the dorsal dislocation of the ulnar head. CONCLUSION: This technique seemed to be safe and demonstrated good results in these four wrists. It should be proposed in patients with Madelung deformity and assessed prospectively.     

Results of the operative treatment in Madelung's deformity]

Nine wrists in eight patients were treated surgically between 1988 and 2003 for symptomatic Madelung's deformity. The pain of involved wrist followed by forearm deformation fulfilled criteria for surgery. Closing wedge osteotomy of the distal radius were carried out eight times accompanied by shortening of the ulna (four patients), excision of the distal ulna (one patient), and no ulnar surgery (three patients). Pain relieved after surgery. The follow up period ranged from 1 to 9.5 years. No pour results were stated in subjective patient's estimation during final check up. Wrist appearance were stated to be satisfactory. Limitation of the range of motion concerning supination and pronation of the forearm were stated invariably. X-ray retrospective assessment of the inclination angle, lunate coverage and presence of arthritic changes were conducted. Time and method of surgical treatment for Madelung's deformity should be considered individually.     

Isolated wedge osteotomy of the ulna for mild Madelung's deformity

PURPOSE: Madelung's deformity is a characteristic pattern of anterior-ulnar bowing of the radius and a dorsally prominent ulnar head. Even if this deformity is associated with a certain degree of functional impairment, patients are satisfied with their function and mainly complain about the appearance of their wrists. The purpose of this study was to report a new surgical procedure (shortening combined with a slight anterior angulation osteotomy of the ulna) aiming to improve the appearance of the wrist and to relieve pain if present without compromising the function of the wrist. This technique is suitable for mild cases of Madelung's deformity. METHODS: This is a retrospective study of 4 wrists in 3 patients. All patients had a mild form of Madelung's deformity (without any dislocation of the lunate). Even if it was not their primary motivation to have surgery, all of the patients preoperatively experienced some wrist pain. An anterior angulation and shortening osteotomy of the ulna shaft was performed through a dorsal medial approach and fixed with a dynamic compression plate. RESULTS: At 24 months follow-up, all of the patients were satisfied with the appearance of their wrists and forearms. The distal radioulnar joint was congruent radiologically in all cases, and the range of active pain-free forearm rotation improved. CONCLUSIONS: This technique seems to be safe and reliable in mild cases of Madelung's deformity.     

Childhood nonunion of ulna presenting with wrist deformity in an adult: a case report

We describe an adult patient with traumatic, nonunion of ulna sustained at 11 years of age who presented with wrist deformity. The possible pathogenesis, differential diagnoses and its successful management are described. A 23-year- old right hand dominant male presented with a progressive wrist deformity of his right upper limb. At 11 years of age, he sustained an isolated open fracture of the right forearm. He had nonoperative treatment. He had 60 degrees of ulnar deviation at wrist. He had no pain in the wrist or elbow. He was able to do all activities using his right upper limb. Radiograph revealed a nonunion of ulna in mid-shaft. The radius was bowed. Radiographs at the time of injury revealed a displaced both bones forearm fracture in mid-shaft. He underwent open reduction, internal fixation of ulna with bone grafting and a corrective osteotomy of the radius. The contracted Extensor carpi ulnaris was Z lengthened. Seven months postoperative, both the nonunion of ulna and radius osteotomy were consolidated. The wrist had no deformity. He had returned to preoperative activity level. Though nonunion is rare in pediatric forearm fractures, asymmetric bone and soft tissue growth can lead to deformities even in the absence of physeal injury. In addition to the standard treatment of nonunion, maintenance of the relative lengths of radius and ulna is essential, to obtain optimum function.