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Madelung’s deformity is an uncommon congenital wrist condition characterized by premature closure of the volar-ulnar aspect of the distal radius physis, volar carpal subluxation, and distal ulna prominence. It is classified as a malformation of the radio-ulnar axis involving the entire upper limb, according to the Oberg-Manske-Tonkin Classification. It accounts for less than 2% of congenital upper extremity differences. Madelung’s deformity is most commonly idiopathic, but a Madelung-like deformity may result from trauma, infection, multiple hereditary exostoses (MHE), and Ollier’s disease. Additionally, it is associated with skeletal dysplasias involving mutations of the short stature homeobox (SHOX) gene. Madelung’s deformity predominantly affects females and becomes clinically apparent during adolescence. Affected individuals may present with wrist pain, restricted range of motion of the wrist and forearm, decreased grip strength, and function difficulties, as well as aesthetic concerns. Several surgical options have been described for children with Madelung’s deformity, depending on their age and degree of deformity, and include physiolysis, soft tissue release, and osteotomies, with promising outcomes.

The management of the hypoplastic thumb depends on the degree of bony malformation, soft tissue anomaly, and deficiencies. Type IIIA thumbs have thumb-index web-space narrowing, hypoplasia of the thenar musculature, metacarpophalangeal joint instability, and extrinsic tendon abnormalities. They also have a hypoplastic metacarpal with a stable carpometacarpal joint, differentiating them from type IIIB thumbs which have an unstable carpometacarpal joint due to partial metacarpal aplasia. Whereas type IIIB thumbs are best treated with ablation and index pollicization, type IIIA thumbs are amenable to reconstruction. Many techniques have been described for correction of the variety of deformities found in theses thumbs. The purpose of this article is to present our preferred technique for comprehensive reconstruction of type IIIA thumbs.

Purpose: Children with radial longitudinal deficiency have very short forearms. The Ilizarov method of distraction osteogenesis has been used to increase extremity length, but results differ with the underlying condition and the limb being lengthened. The purpose of this study is to examine retrospectively the outcomes of Ilizarov lengthening of the ulna in children with radial longitudinal deficiency. Methods: Nine children with radial longitudinal deficiency had 13 ulnar lengthenings using the Ilizarov method; 8 with unilateral deficiency had 9 lengthenings to improve appearance, and 1 child with bilateral radial longitudinal deficiency had 4 lengthenings to improve function. All had previous wrist centralization surgery, and all but 1 had a carpal osteotomy at the time of lengthening to reduce residual wrist angulation. Results: The average gain for each lengthening was 4.4 cm (range 1.8‐8.0 cm) with an average lengthening index of 9 weeks per cm (range 4‐24 weeks per cm). All patients at each lengthening experienced at least 1 pin site infection that required antibiotic treatment. Other complications included delayed union requiring internal fixation and bone grafting in 3 lengthenings and recurrence of radial deviation requiring shortening and wrist arthrodesis in 1 case. Conclusions: The Ilizarov technique is an effective method for lengthening the ulna in children with radial longitudinal deficiency. The process of lengthening is prolonged and arduous with frequent complications. In successful cases, however, patients are pleased with the function and appearance of their lengthened forearms. (J Hand Surg 2007;32A: 1402‐1407. Copyright © 2007 by the American Society for Surgery of the Hand.)

Background: Posttraumatic and congenital forearm deformities in children can be difficult to appreciate in all planes. In cases of distal radioulnar joint instability and loss of forearm rotation, surgical correction is challenging. Advances in 3-dimensional printing allow creation of custom guides at a reasonable cost, enabling precise correction of the deformity in all planes. Methods: Nineteen children with deformity of the forearm had corrective osteotomies performed using preoperative 3-dimensional computer modeling and patient-specific surgical guides. Surgicase software was used for 3-dimensional planning of the corrective osteotomy, by superimposing a mirror image of the unaffected side as a template. Based upon this planning, patient-specific surgical guides were manufactured. Radiographic and clinical outcomes were assessed. Results: Three patients had a diagnosis of multiple hereditary exostoses, and one of Madelung’s deformity. The remaining 15 patients had a diagnosis of fracture malunion. Average preoperative angulation of both the radius and ulna was 23 degrees. For the patients with fracture malunions, the time from injury to surgery ranged from 6 months to 8 years. Twelve patients underwent osteotomies of both the radius and ulna, 5 had osteotomies of the radius alone, and 2 had a single osteotomy of the ulna only. All osteotomies went on to unite and no patient lost range of motion. Preoperative arc of forearm rotation averaged 101 degrees (range 0 to 180 degrees). Postoperatively, this improved to 133 degrees (range 85 to 180 degrees). Eight patients had distal radioulnar instability preoperatively, all of which normalized after surgery. There were 4 complications: 1 hypertrophic scar, 1 subject with extensor pollicis longus weakness, and 2 transient sensory losses in the superficial radial nerve distribution. Conclusions: This case series demonstrates that 3-dimensional computer modeling permits complex and multiple osteotomies to be done safely to achieve deformity correction in children. Limitations in forearm rotation and distal radioulnar malalignment can be reliably improved using this technique. Level of Evidence: Level IV—retrospective case series.

The abductor digiti minimi is one of several muscles described as transfers for restoration of thumb opposition, a critically important function. This operation, also known as the Huber opponensplasty, is most commonly used for reconstruction of opposition in children with type 2 or 3a thumb deficiency, when it is often combined with Z-plasty of the thumb-index web space and metacarpophalangeal ulnar collateral ligament reconstruction. In our experience this operation reliably improves thumb opposition for children with this thumb deficiency.

Expansion of the discipline of hand surgery and heightened interest in congenital problems have resulted in major advances in the treatment of congenital hand anomalies over the past 25 years. Increased experience with congenital anomalies of the hand has expanded the hand surgeon's knowledge of patterns and relationships between different anomalies resulting in new methods of classification and more logical approaches to treatment. The principles of treatment of the more common anomalies, such as syndactyly, established by prior generations of hand surgeons have been refined in details of technique. New technologies, such as distraction lengthening and free vascularized transfers, have allowed the surgeon to treat new problems and old problems in new ways. In spite of our successes, much remains to challenge hand surgeons in this new millennium, especially in the construction of joints and the expanding field of fetal surgery.

The records of 119 patients with 196 extremities with radial longitudinal deficiency seen between 1923 and 1996 were reviewed. We propose a global classification system that includes the spectrum of pathology affecting the radial side of the extremity, including deficiency of the radius, carpal abnormalities, and hypoplastic thumbs. Radial deficiency could be classified for 181 extremities of 104 patients using this classification system. Type N has a normal length radius and a normal carpus with thumb hypoplasia, type O has a normal length radius and radial side carpal abnormalities, type 1 has more than 2 mm shortening of the radius, type 2 has a hypoplastic radius, type 3 has a partial radius with absence of the distal physis, and type 4 has complete absence of the radius. All patients had thumb hypoplasia. Eighty-two percent of extremities with thumb hypoplasia but no deficiency of the radius that were available for carpal bone classification had carpal anomalies, including absence, hypoplasia, and coalitions. All the extremities with type 1 radial deficiency had carpal anomalies. Carpal abnormalities could not be determined for types 2, 3, and 4 deficiency because most had a prior centralization. Proximal radioulnar synostosis or congenital dislocation of the radial head was seen in 44% of extremities with type 1 radial deficiency. This classification includes carpal anomalies and thereby links isolated thumb hypoplasia and deficiency of the radius into one system.

The records of 98 patients with 160 hypoplastic thumbs seen between 1923 and 1993 were reviewed to determine the salient characteristics of this population. Physical findings, photographs, and x-ray films were studied. Sixty-three percent of patients were male, and 63% had bilateral thumb hypoplasia, 59% had radial dysplasia, and 86% had other anomalies. An associated syndrome, most commonly the vertebral, anal, tracheosophageal, renal, and radial limb anomalies association or Holt-Oram syndrome, was present in 44%. Patients with spine, genitourinary, or gastrointestinal anomalies were most likely to have the vertebral, anal, tracheosophageal, renal, and radial limb anomalies association, those with cardiac anomalies were most likely to have Holt-Oram syndrome, and those with lower extremity anomalies were most likely to have a different syndrome. One hundred thirty-nine thumbs were classifiable, using a modification of Blauth's classification; 19% were types 1 and 2, 23% were type 3, and 58% were types 4 and 5. One hundred seven operations were performed on 63 upper limbs, including 24 thumb reconstructions and 35 pollicizations. These patients must be carefully examined for bilaterality, other anomalies, and syndromes. Classification using a modification of Blauth's criteria is useful and helps the surgeon determine a treatment plan.

Madelung's deformity results from arrested or slowed growth of the volar-palmar portion of the distal radial epiphysis. The deformity is characterized by increased volar and ulnar tilt of the articular surface of the radius and a bow of the distal radius in a palmar direction. When Madelung's deformity produces pain and interferes with function, correction of the deformity of the radius and secondary overgrowth of the ulna is desirable. Although osteotomy of the radius near the junction of the middle and distal thirds allows correction of the bow of the radius and limited correction of palmar tilt, it does not permit correction of ulnar tilt. We describe a very distal radial osteotomy that permits correction of the 3 deformities of the distal radius independently; correction of palmar tilt, ulnar tilt, and the bow of the radius are selected individually. The inherent flexibility makes the procedure technically challenging but allows for maximum independent correction of the components of Madelung's deformity. Early results indicate the procedure provides relief of pain and improved appearance with few complications.

Purpose Although the x-ray changes of advanced Madelung's deformity are well described, little quantitative information exists on the subtle, early changes of the deformity. This study investigated the threshold of 4 radiographic measurements that predict a diagnosis of Madelung's deformity by experienced hand surgeons. Methods Ulnar tilt, lunate subsidence, lunate fossa angle, and palmar carpal displacement were measured on x-rays of patients with Madelung's deformity. For each set of x-rays 4 opinions were available concerning a diagnosis of Madelung's deformity as based on the x-rays only. The 4 quantitative measurements were examined to determine a threshold value that predicts a unanimous diagnosis of Madelung's deformity. Results The thresholds for the diagnosis of Madelung's deformity are: ulnar tilt of 33° or greater, lunate subsidence of 4 mm or more, lunate fossa angle of 40° or greater, and palmar carpal displacement of 20 mm or more. When any one of these measurements equaled or exceeded this threshold value, the raters were unanimous in establishing a diagnosis of Madelung's deformity. Conclusions Threshold values of ulnar tilt, lunate subsidence, lunate fossa angle, and palmar carpal displacement for the diagnosis of Madelung's deformity are determined. Lunate fossa angle may prove especially useful in the diagnosis of early Madelung's deformity. Type of study/level of evidence Diagnostic I.

Purpose To evaluate 5 defined measurement techniques that are applicable to x-rays of Madelung's deformity: ulnar tilt, lunate subsidence, lunate fossa angle, palmar tilt, and palmar carpal displacement. The measurements rely on the longitudinal axis of the ulna and the carpal bones to determine drawing lines and avoid the distorted distal radius and its deformed lunate fossa. The reliability and reproducibility of the measurements is determined. Methods Forty-eight sets of posteroanterior and lateral x-ray views of the wrist of subjects with the clinical diagnosis of Madelung's deformity were measured by 4 raters. Each rater made the 5 defined measurements on each pair of x-rays. Pairs of raters were compared for reliability using the Pearson correlation coefficient and Lin's concordance correlation coefficient. Two raters repeated the 4 reliable measurements a minimum of 6 months after the first measurements. Each rater's results were compared for reproducibility using Lin's concordance correlation coefficient. Results Ulnar tilt and lunate subsidence have excellent reliability and reproducibility. Palmar carpal displacement has acceptable reliability and reproducibility. Lunate fossa angle has borderline reliability but excellent reproducibility. Palmar tilt has poor reliability. Conclusions Ulnar tilt, lunate subsidence, and palmar carpal displacement, as defined, are considered reliable and reproducible measurements for quantifying the severity of Madelung's deformity on x-rays. Lunate fossa angle is not sufficiently reliable for comparing preoperative and postoperative wrists but may prove useful in establishing an early diagnosis. Palmar tilt is not measured reliably on a lateral x-ray because of the superimposition of multiple structures on a lateral x-ray and the absence of the volar part of the lunate fossa in patients with severe Madelung's deformity. Advanced imaging techniques are needed to delineate the deformity of the distal radius in a lateral projection. Type of study/level of evidence Diagnostic, Level II.

Background: Congenital longitudinal deficiencies of the radius and thumb are known to be associated with one another; however, the details of their relationship are unknown. The purpose of this study was to determine whether increased severity of radial deficiencies is associated with increased severity of thumb deficiencies and to review the relationship between radial deficiency and reconstructibility of a hypoplastic thumb. Methods: Radiographs and charts of 227 affected upper extremities of 139 patients with radial longitudinal deficiency were reviewed. The associated thumb deficiency was classified according to a modification of the Blauth and Schneider-Sickert scheme and the radial deficiency was classified according to a modification of the Bayne and Klug criteria for 191 extremities of 119 patients. Results: The severity of the thumb deficiency was directly proportional to the severity of the radial deficiency (p < 0.0001). Half of the extremities had either a thumb deficiency or thumb and carpal deficiencies without radial deficiency. Two-thirds (sixty-three) of the ninety-five limbs with a normal radius had a thumb that could be surgically reconstructed. Seventy-one (91%) of seventy-eight extremities with a thumb amenable to surgical reconstruction had a radius that did not require surgical reconstruction. All extremities with a radial and/or carpal deficiency had a thumb deficiency. Forty-eight (94%) of fifty-one extremities with complete absence of the radius had a thumb that was not reconstructible. Conclusions: This study supports the growing body of evidence that the components of radial longitudinal deficiency represent a progressive spectrum of upper extremity abnormalities, and a distal progression of severity, with distal structures likely to be more involved than proximal structures. Level of Evidence: Prognostic study, Level II-1 (retrospective study). See Instructions to Authors for a complete description of levels of evidence.

Twenty patients underwent proximal row carpectomy and were retrospectively evaluated for pain, motion, grip strength, functional activity, and x-ray changes at a mean follow-up of 3 1/2 years. For nonrheumatoid patients, motion decreased 15% after surgery, mean grip strength improved 22%, and 82% believed their conditions were improved and said they would repeat the procedure. The procedure failed in all three patients with rheumatoid arthritis. Patients with mild preoperative arthritic changes had better results than those with advanced disease.

The flexor digitorum superficialis is a digastric muscle with a proximal muscle belly from which the tendons to the index, ring and little fingers arise. These tendons are not independent and are not good motors for non-synergistic transfers. Such as for finger extension. The muscle and tendon to the middle finger arise separately and are therefore more suitable for non-synergistic transfers.

Paralytic supination deformity of the forearm, secondary to obstetrical palsy, was treated by biceps tendon rerouting in 11 children. In two patients additional pronation was obtained by subsequent percutaneous osteoclasis and rotation of the radius and ulna. Correction of the supination deformity resulted in improvement of single-handed as well as two-handed activities in all patients.

To determine the course of the so-called mucous cyst both after various modalities of treatment and without treatment, 56 patients with 62 cysts were contacted to participate in a study with an average follow-up of 6.2 years after initial evaluation. The mucous cysts primarily occurred in elderly women and were most prevalent in the index and long fingers. Longitudinal grooving of the nail was not uncommon and may have preceded the appearance of the cyst. Degeneration of the distal interphalangeal joint was frequently found. The long-term results after treatment with simple aspiration or decapping, or by excision with skin grafts and flaps as needed or with observation only were similar. However, surgical excision or aspiration may eliminate earlier the cosmetic disfigurement and occasional discomfort that are associated with this lesion.

To compare the results of epineurial and intrafascicular perineurial nerve repair techniques, ulnar nerves of 20 domestic cats were severed proximal to the medical humeral epicondyle. One nerve was repaired with No.8-O nylon by standard epineurial technique with the aid of three to five magnifications; the other nerve was repaired with No. 10-O nylon by intrafascicular perineurial technique under higher magnification. Four to five months following neurorrhaphies, the ambulation pattern, fanning of claws, and sensation were evaluated subjectively. Objective measurements included efficiency, absolute strength, and exact weight of the flexor carpi ulnaris muscle. Proximally and distally to each neurorrhaphy, complete axon counts were taken. Histochemically and histologic analyses were made on reinnervated muscles and neuromas. No statistically significant differences were detected between the results of epineurial and intrafascicular perineurial neurorrhaphies. These findings indicate that, following acute nerve laceration in the cat, epineurial neurorrhaphy is as satisfactory as is intrafascicular perineurial neurorrhaphy.

A group of 49 patients with 74 injured digital nerves was studied more than 5 years following epineurial nerve suture. Three tests of return of sensibility were used: von Frey plastic filaments, a modified Weber two-point discrimination device, and a dynamic test--the plastic ridge. The correlation between two-point discrimination and tactilegnosis in the range of 8 to 12 mm was poor. The plastic ridge device detects the presence or absence of tactilegnosis in patients in the intermediate range of two-point discrimination between 8 and 12 mm. Of the factors studied, the patient's age at the time of nerve suture was found to be directly related to the return of sensibility by both two-point discrimination and ridge criteria.

Satisfactory results were obtained in 20 of 21 abductor digiti minimi opponensplasties performed in children with congenital radial dysplasia. A modified technique left the muscle attached to the pisiform and did not require complete dissection of the neurovascular structures. An adequate subcutaneous tunnel was made to accept the transferred muscle and laxity of the ulnar collateral ligament of the metacarpal-phalangeal joint of the thumb, a consistent finding in patients with isolated aplasia of the abductor pollicis brevis and opponens pollicis muscles, was repaired by imbrication and suture of one of the tendinous slips to the ulnar capsule.

Epineurial neurorrhaphy under tension and interfascicular grafting, two methods of bridging gaps associated with loss of a nerve segment, were compared. After resection of a 2 cm length of both ulnar nerves in cats, one nerve was sutured under tension using an epineurial technique, and the other was repaired using multiple interfascicular sural nerve grafts. Six months later return of nerve function was evaluated. Subjective evaluation included ambulation, sensation, and intrinsic function. Objective measurements included muscle efficiency, maximum strength, muscle weights, and total axon counts. No statistical difference was observed between these two techniques. Histochemically, there was marked fiber type grouping of the reinnervated muscles for both types of repairs. Histologically, perineurial fibrosis and axonal disorganization were equal for both techniques, but significantly greater suture granuloma formation occurred in the nerve repaired under tension. Individual grafts retained their identity and remained distinct grossly and microscopically.

Although nerve injury rarely accompanies wrist trauma or disease, the long-term functional result frequently depends more on resolution of the nerve deficit and symptoms than on resolution of the basic orthopedic pathology. The origins of nerve injury associated with orthopedic problems are reviewed, and a rational approach to their management is described.

An attempt has been made to enumerate or describe briefly the various clinical manifestations which may be associated with congenital neurofibromatosis. In some instances the direct relationship can be proved, but in the majority of cases this has to be assumed. The frequency with which these various questionable manifestations are associated with unmistakable signs of neurofibromatosis, however, and the tissue characteristics which are found so commonly in all cases of soft-tissue hypertrophy, represent strong presumptive evidence that all may arise from a common etiology,—a primary developmental defect of the nervous system.

Centralization arthroplasty via a transverse ulnar approach was used to correct radial clubhand deformity in 21 children. The patients were evaluated quantitatively by measurements of hand-forearm angle and hand-forearm position.

Two skeletally mature baboons underwent comparative neurorrhaphies following transsection of both ulnar nerves without loss of nerve tissue. In baboon 1 there was no difference in objective evaluations at 12 months when an epineurial neurorrhaphy was compared to a perineurial fascicular neurorrhaphy. In baboon 2, the one that had interfascicular grafts in one upper extremity and an epineurial neurorrhaphy on the other side, there was significantly better functional return on the epineurial side than on the graft side. Gross and histological evaluation showed that the grafts retained their integrity and regenerating axons transversed the grafts well.

Preiser's disease, or avascular necrosis of the scaphoid, is an exceedingly rare although debilitating pathologic process. A case of progressive avascular necrosis of the carpal scaphoid is presented together with a review of the possible etiology of this rare syndrome. Histologic evaluations of resected specimens have demonstrated a localized sequestrum of necrotic bone, leading to the belief that disruption of blood supply is a major predisposing factor. The diagnosis of Preiser's disease or avascular necrosis of the carpal scaphoid should be exclusively reserved to those cases that demonstrate a progressive clinical and radiographic sequence of osteosclerosis and subsequent fragmentation of the scaphoid.

There is usually a high incidence of injuries to the musculoskeletal system in the severely injured patient. These injuries to the musculoskeletal system should be protected temporarily by adequate pressure dressings, splinting, or traction until more definitive treatment can be instituted. The presence of a compound fracture represents a true surgical emergency. However, in the presence of serious injuries to other parts of the body, even the compound fracture may have to await treatment of the other injuries before definitive therapy can be instituted. The severely injured patient should receive the team concept of management with the one most appropriate physician serving as team commander.