Ann E. Van Heest

Arthrogryposis: a review and update.

Congenital contractures can be divided into two groups: isolated contractures and multiple contractures (Fig. 1). Isolated congenital contractures affect only a single area of the body; the most common isolated contracture is congenital clubfoot, which occurs in one of every 500 live births1. Fig. 1 Types of congenital contractures. The term arthrogryposis is often used as shorthand to describe multiple congenital contractures that affect two or more different areas of the body. Arthrogryposis is not a specific diagnosis, but rather a clinical finding, and it is a characteristic of more than 300 different disorders2,3. The overall prevalence of arthrogryposis is one in 3000 live births4. The inheritance, natural history, treatment guidelines, and outcomes of arthrogryposis vary among disorders, underscoring the importance of making a specific diagnosis in each child1,5-10. The purpose of this article is to present the current state of knowledge about the classification, etiology, and management of children with various types of arthrogryposis. To establish a differential diagnosis, it is important to first decide whether a child has normal neurological function. A normal neurological examination suggests that arthrogryposis is due to amyoplasia, a distal arthrogryposis, a generalized connective tissue disorder, or fetal crowding. In contrast, an abnormal neurological examination indicates that movement in utero was diminished as a result of an abnormality of the central or peripheral nervous system, the motor end plate, or muscle. ### Amyoplasia Amyoplasia (A = no; myo = muscle; plasia = growth) is a distinct form of arthrogryposis with characteristic clinical features as shown in Figure 2: the shoulders are usually internally rotated and adducted, the elbows are extended, the wrists are flexed and ulnarly deviated, the fingers are stiff, and the thumbs are positioned in the palm. In the lower limbs, …

Sensibility deficiencies in the hands of children with spastic hemiplegia

We evaluated 40 children with spastic hemiplegia due to cerebral palsy for sensory function and relative limb size in the affected and unaffected upper extremities. Sensory function of each limb was evaluated with respect to stereognosis (12 objects), two-point discrimination, and proprioception. Four size measurements of each limb were made: arm and forearm circumference and forearm and forearm-hand length. This study showed that 97% of the spastic limbs had a stereognosis deficit, 90% had a two-point discrimination deficit, and 46% had a proprioception deficit. Thus sensory deficits are the rule rather than the exception in children with spastic hemiplegia. Those children with severe stereognosis deficits had significantly smaller limbs in all four measurement parameters than the children with mild or moderate stereognosis deficits. In the preoperative evaluation of children with spastic hemiplegia, severe size discrepancy is a physical examination tool that can be used as a predictor of severe sensory deficits. This information is helpful for the hand surgeon in establishing realistic surgical goals.

Surgical treatment of carpal tunnel syndrome and trigger digits in children with mucopolysaccharide storage disorders.

The role of surgical intervention for carpal tunnel syndrome (CTS) and trigger digits in children with mucopolysaccharide storage disorders (MPSDs) has not been clearly defined, particularly as the treatment of the underlying disease has advanced to include bone marrow transplantation. This study reviews our experience in the treatment of CTS and trigger digits in 22 children with MPSDs who were evaluated for CTS by electromyographic (EMG)/nerve conduction velocity (NCV) testing. Seventeen children were diagnosed with CTS by EMG/NCV testing and were treated with bilateral open surgical release with or without flexor tenosynovectomy. The EMG/NCV testing revealed normal results in 5 patients who are subsequently being monitored. Forty-five digits in 8 children were diagnosed clinically with trigger digits. Nineteen digits were treated by annular pulley release alone. Twenty-six digits were treated by annular pulley release with partial flexor digitorum superficialis tendon resection. The average age at the time of hand surgery was 6.3 years, and at the time of follow-up, 9.6 years. Postoperative EMG/NCV testing in 7 children showed 1 with improvement and 6 with normalization. None of the patients undergoing carpal tunnel release went on to develop thenar atrophy or absent sensibility, as has been reported in untreated cases. Patients were evaluated for triggering digits both by preoperative tendon palpation and by intraoperative flexor tendon excursion at the time of open carpal tunnel release. All patients undergoing trigger release had improved active digital flexion seen at the final follow-up visit. Because of the very high incidence of CTS and trigger digits in this population, the authors currently recommend routine screening of EMG/NCV for all children with MPSDs. Early surgical intervention for nerve compression and stenosing flexor tenosynovitis can maximize hand function in these children.

Thumb carpal metacarpal arthritis.

Abstract The thumb carpometacarpal (CMC) joint is the most common site of surgical reconstruction for osteoarthritis in the upper extremity. In patients older than age 75 years, thumb CMC osteoarthritis has a radiographic prevalence of 25% in men and 40% in women. The thumb CMC joint obtains its stability primarily through ligamentous support. A diagnosis of thumb CMC arthritis is based on symptoms of localized pain, tenderness and instability on physical examination, and radiographic evaluation. A reproducible radiographic classification for disease severity is based on the fourstage system described by Eaton. Nonsurgical treatment options include hand therapy, splinting, and injection. Surgical treatment is tailored to the extent of arthritic involvement and may include ligament reconstruction, metacarpal extension osteotomy, arthroscopic partial trapeziectomy, implant arthroplasty, and trapeziectomy with or without ligament reconstruction and tendon interposition.

Intrinsic balancing in reconstruction of the tetraplegic hand

This article reviews 183 hand reconstructions in 135 consecutive tetraplegic patients. Comparisons were made between 103 extrinsic reconstructions with intrinsic balancing procedures and 80 extrinsic reconstructions without intrinsic balancing procedures. Extrinsic reconstructions (tendon transfers and tenodesis in the forearm muscles) were augmented by intrinsic reconstructive procedures (tendon transfers or tenodesis to improve the intrinsic balance of the fingers) in patients exhibiting digital imbalance. Intrinsic procedures included primarily the flexor digitorum superficialis (FDS) lasso procedure or the intrinsic tenodesis procedure. The patients were stratified by level of spinal cord injury and by type of extrinsic and intrinsic reconstruction. Hands reconstructed with intrinsic balancing versus without intrinsic balancing, as well as intrinsic balancing using a FDS lasso procedure versus an intrinsic tenodesis procedure, were compared with patients with the same level of spinal cord function. Patients who underwent reconstructions with intrinsic balancing had more grip strength, by an average of 13-26 N, than those who did not undergo intrinsic balancing. When different intrinsic procedures were compared, there was improvement in grip strength and function in activities of daily living for all hands, but there was no significant difference between FDS lasso or intrinsic tenodesis procedures. The indications for intrinsic balancing during extrinsic reconstruction are developed into treatment algorithms based on the senior authors surgical experience. The authors recommended that digital intrinsic procedures be included in hand reconstruction for tetraplegic patients exhibiting intrinsic imbalance to help improve digital function and provide increased grip strength.

Assessment of Technical Skills of Orthopaedic Surgery Residents Performing Open Carpal Tunnel Release Surgery

BACKGROUND Motor skills assessment is an important part of validating surgical competency. The need to test surgical skills competency has gained acceptance; however, assessment methods have not yet been defined or validated. The purpose of the present study was to evaluate the reliability and validity of four testing measures for the integrated assessment of orthopaedic surgery residents with regard to their competence in performing carpal tunnel release. METHODS Twenty-eight orthopaedic residents representing six levels of surgical training were tested for competence in performing carpal tunnel release on cadaver specimens. Four measures were used to assess competency. First, a web-based knowledge test of surgical anatomy, surgical indications, surgical steps, operative report dictation, and surgical complications was administered. Second, residents participated in an Objective Structured Assessment of Technical Skills; each resident performed surgery on a cadaver specimen. All residents were evaluated independently by two board-certified orthopaedic surgeons with a subspecialty certificate in hand surgery with use of a detailed checklist score, a global rating scale, and a pass/fail assessment. The time for completion of the surgery was also recorded. Each assessment tool was correlated with the others as well as with the residents level of training. RESULTS Significant differences were found between year of training and knowledge test scores (F = 7.913, p < 0.001), year of training and detailed checklist scores (F = 5.734, p = 0.002), year of training and global rating scale (F = 2.835, p = 0.040), and year of training and percentage pass rate (F = 26.3, p < 0.001). No significant differences were found between year of training and time to completion of the carpal tunnel release (F = 2.482, p < 0.063). CONCLUSIONS The results of the present study suggest that both knowledge and cadaver testing discriminate between novice and accomplished residents. However, although failure of the knowledge test can predict failure on technical skills testing, the presence of knowledge does not necessarily ensure successful performance of technical skills, as cognitive testing and technical skills testing are separate domains.

Surgical treatment of arthrogryposis of the elbow

The purpose of this study was to analyze our results of surgical treatment of arthrogryposis of the elbow and to compare our tendon transfer results using range of motion (ROM) criteria versus functional use criteria. Eighteen tendon transfers for elbow flexion in 14 children with arthrogryposis with an average follow-up period of 4 years (range, 1-14 years) and 6 elbow capsulotomies with triceps lengthening in 6 children with arthrogryposis with an average follow-up period of 5 years (range, 2-9 years) were evaluated. Each child was assessed by a questionnaire regarding functional use of the upper extremity, physical examination of ROM and strength, and a videotaped activities of daily living evaluation. Tendon transfer results were classified and compared using 2 methods of evaluation: postoperative strength and ROM and effective functional use of the tendon transfer to perform activities of daily living. The 6 elbow capsulotomies improved from an average preoperative arc of 17 degrees of motion (average extension, -2 degrees; average flexion, 19 degrees) to an average final follow-up arc of 67 degrees (average extension, -25 degrees; average flexion, 92 degrees). The 18 tendon transfers evaluated by strength and ROM criteria showed 9 triceps to biceps transfers in 9 arms (7 good, 1 fair, and 1 poor), 5 pectoralis to biceps transfers in 4 arms (1 good, 3 fair, and 1 poor), and 4 latissimus dorsi to biceps transfers in 3 arms (2 good and 2 fair). Evaluation by functional use criteria gave the same result in 13 transfers and downgraded the result in 5; the downgraded results were due to resultant flexion contracture or limited functional use because the transfer was in the nondominant arm. Based on this review, optimal surgical candidates for tendon transfer are children older than 4 years, who have full passive ROM of the elbow in the dominant arm, and at least grade 4 strength of the muscle to be transferred.

Development and Validation of a Basic Arthroscopy Skills Simulator

PURPOSE The purpose of our study was to develop a low-fidelity surgical simulator for basic arthroscopic skills training, with the goal of creating a pretrained novice ready with the basic skills necessary for all joint arthroscopic procedures. METHODS A panel of education, arthroscopy, and simulation experts designed and evaluated a basic arthroscopic skills training and testing box. Task deconstruction was used to create 2 modules, which incorporate core skills common to all arthroscopic procedures. Core metrics measured were time to completion, number of trials to steady state, and number of errors. Face validity was evaluated using a questionnaire. Construct validity was examined by comparing 8 medical students with 8 expert orthopaedic surgeons. RESULTS Surgeons were faster than students on both module 1 (P = .0013), simulating triangulation skills, and module 2 (P = .0190) simulating object manipulation skills. Surgeons demonstrated fewer errors (6.9 errors versus 28.1; P = .0073). All surgeons were able to demonstrate steady state (i.e., perform 2 trials that were within 10% of each other for time to completion and errors) on both modules within 3 trials on each module. Only 2 novices were able to demonstrate steady state on either module, and both did so within 3 trials. Furthermore, face validity of the skills trainer was shown by the expert arthroscopists. CONCLUSIONS We describe a basic arthroscopy skills simulator that has face and construct validity. Our expert panel was able to design a simulator that differentiated between experienced arthroscopists and novices. CLINICAL RELEVANCE Surgical simulation is an important part of efficient surgical education. This simulator shows good construct and face validity and provides a low-fidelity option for teaching the entry-level arthroscopist.

Posterior Elbow Capsulotomy with Triceps Lengthening for Treatment of Elbow Extension Contracture in Children with Arthrogryposis

BACKGROUND Flexion of one elbow is essential to enable children with arthrogryposis to achieve independent function such as self-feeding and self-care of the face and hair. We analyzed the outcomes of posterior elbow capsulotomy with triceps lengthening for the treatment of elbow extension contractures in a series of children with arthrogryposis multiplex congenita. METHODS The medical records of all children with arthrogryposis who had been followed for a minimum of two years after treatment with elbow capsulotomy and triceps lengthening were retrospectively reviewed. The postoperative range of motion and ability to reach the mouth were compared with the preoperative status. RESULTS Posterior capsulotomy with triceps lengthening was performed in twenty-nine elbows of twenty-three children with an average age of thirty-five months (range, seven months to thirteen years). The average duration of follow-up was 5.4 years. The arc of motion of all twenty-nine elbows improved from an average of 32 degrees (range, 0 degrees to 75 degrees) preoperatively to an average of 66 degrees (range, 10 degrees to 125 degrees) at the time of final follow-up. All children were able to reach the mouth using passive assistance (e.g., table-push, trunk-sway, and cross-arm techniques), and twenty-two children were able to feed themselves independently. No child underwent subsequent tendon transfer surgery. CONCLUSIONS Elbow capsulotomy with triceps lengthening successfully increases passive elbow flexion and the arc of elbow motion of children with arthrogryposis, enabling hand-to-mouth activities. In contrast to studies in which tendon transfer surgery was used to increase elbow flexion, none of the children in this series underwent subsequent tendon transfer surgery.

Acute forearm lengthenings.

We describe our surgical technique of acute pediatric forearm lengthening and joint leveling for treatment of symptomatic forearm-length discrepancies. A retrospective clinical and radiographic analysis was performed of all patients undergoing acute forearm lengthenings of > 1.0 cm between 1983 and 1993. Twenty-four acute forearm lengthenings were reviewed with an average follow-up of 3 years. The diagnosis included osteochondromatosis in 17 patients, growth arrest in four patients, and skeletal dysplasia in three patients. Surgical indications included progressive forearm or wrist deformity, significantly limited or painful forearm rotation, or radial-head subluxation. The average lengthening was 1.5 cm (range, 1.0-2.3), which was 9% of total length (range, 3-20%). The goal for lengthening and wrist-joint leveling was near-neutral ulnar variance and was achieved in all cases. We conclude that the forearm can be lengthened acutely successfully to achieve near-neutral ulnar variance in children with forearm-length discrepancies caused by osteochondromas, growth arrests, or bone dysplasias. The surgical technique and the results are described in 24 forearm lengthenings.